Josephine Isabel-Jones

Partial Fontan: Advantages of an adjustable interatrial communication☆

Systemic venous hypertension after the Fontan procedure is a major cause of mortality and morbidity, accounting for 11 of 16 deaths in our series of 228 Fontan procedures. A partial Fontan with a residual atrial septal defect (ASD) would allow controlled right-to-left shunting to reduce venous pressure and improve cardiac output while maintaining a reduced but acceptable arterial oxygen saturation. This allows complete or graded closure of the ASD after the discontinuation of cardiopulmonary bypass in the operating room or at any time in the postoperative period by exposing the snare under local anesthesia. From 1987 to 1990, 36 patients undergoing the modified Fontan procedure had placement of an adjustable interatrial communication. Indications for placement of an adjustable ASD included increased pulmonary artery pressures, increased pulmonary vascular resistance, reactive airway disease, previously increased or unknown pulmonary vascular resistance, small pulmonary arteries, and borderline ventricular function. Fourteen patients had the adjustable ASD closed at the time of operation, 8 patients underwent narrowing, and 12 underwent closure of the ASD in the postoperative period. Eight patients were discharged with the ASD partially open, and 2 patients underwent delayed closure. The partial Fontan with an adjustable ASD may increase the safety of the Fontan procedure for high-risk groups such as those with increased pulmonary vascular resistance, pulmonary hypertension, and impaired left ventricular function and for infants, who tolerate venous hypertension poorly. The ability to adjust the ASD in stages depending on the hemodynamic response increases flexibility and safety.

Two-dimensional echocardiographic assessment of left ventricular volumes and ejection fraction in children.

The ability of two-dimensional echocardiography to masure left ventricular volumes and ejection fraction was evaluated in 25 children with congenital heart disease. Dimensions and planimetered areas were obtained in the short-axis view at the mitral valve and high and low papillary muscle levels and in the apical two- and four-chamber views. Eight algorithms using five geometric models were assessed. Left ventricular end-diastolic volume, end-systolic volume and ejection fraction were compared with data from biplane cineangiocardiograms. The correlation varied with the algorithm used. Algorithms using short-axis views appeared superior to those using only apical long-axis views. Four algorithms estimated left ventricular volumes with equal accuracy (Simpsons rule, assuming the ventricle to be a truncated cone; Simpsons rule, assuming the ventricle to be a truncated ellipse; hemisphere cylinder; and ellipsoid biplane). The single algorithm that best estimated left ventricular ejection fraction was the ellipsoid biplane formula using the short-axis view at the papillary muscle level (r= 0.91, slope = 0.94, SEE = 6.7%). Thus, two-dimensional echocardiography can accurately assess left ventricular volumes and ejection fraction in children with congenital heart disease.

Right and left ventricular volume characteristics in children with pulmonary stenosis and intact ventricular septum.

Right (RV) and left ventricular (LV) volume variables were calculated in 27 patients with pulmonary stenosis (PS) during routine cardiac catheterization. These included 21 patients with isolated PS (group 1) and seven studies in six patients (group II) with PS and right-to-left atrial shunt. Right and left ventricular volumes were calculated according to Simpsons rule and the arealength methods respectively.In group 1, right ventricular end-diastolic volume (RVEDV) was not different from normal, RVEF (0.70 ± 0.02) was significantly higher than normal, and right ventricular stroke index (RVSI) (4.36 L/min/M2 ± 0.23) was normal. The RVEDV/LVEDV ratio was significantly less than normal (P 0.001). Multiple regression analysis indicated that RVEDV (% of normal) decreased with both age and severity of RV outflow obstruction (r = 0.77). In group II, RVEDV and RVSI were both less than normal (P < 0.001), while RVEF was normal. LVEDV in the group was slightly higher than normal (P = 0.026) while LVEF was less than normal (P = 0.027) and resulted in normal LVSI.The data suggest that RV and LV function in children with isolated PS are normal, and that knowledge of the RV volume variables is not essential for the management of these patients. In contrast, hearts of patients with PS and right-to-left interatrial shunt have evidence that suggest depressed ventricular function, and the quantitation of RV volume may be helpful in the management of these patients.

Echocardiography reveals a high incidence of bicuspid aortic valve in Turner syndrome

The most common cardiac defect in Turner syndrome has been described previously as coarctation of the aorta. We have evaluated 35 consecutive patients with Turner syndrome by clinical examination and by M-mode and two-dimensional echocardiography. Twelve patients (34%) had isolated, nonstenotic bicuspid aortic valve. A high correlation (82%) existed between the presence of a systolic ejection click and echocardiographic evidence of a bicuspid aortic valve. These data indicate that bicuspid aortic valve may be the most common cardiac anomaly in Turner syndrome.

Factors influencing survival in patients undergoing the bidirectional Glenn anastomosis

The bidirectional Glenn anastomosis (BGA) has long been used as a surgical intervention for patients with single ventricle physiology. Initially, this procedure was the final stage in palliation and was performed in older children. Eventually, as the Fontan procedure came to be used as a method to separate circulations, the Glenn procedure was performed as an intermediate step. Over time, the BGA was performed as an alternative for patients who were considered to be at high risk with the Fontan procedure. Between January 1, 1988, and January 1, 1994, 129 patients underwent BGA at the University of California-Los Angeles. These patients were reviewed retrospectively, including clinic visits, catheterization, and echocardiographic information. The overall survival rate was 87% (112 of 129 patients). The average length of follow-up was 27 months. This information was then analyzed by univariate and multivariate analysis. Several factors were related to failure in patients who underwent BGA including pulmonary artery pressure, systemic right ventricle, and presence of anomolous pulmonary venous drainage and heterotaxy syndrome.

Two-dimensional echocardiographic assessment of right ventricular volume in children with congenital heart disease

The ability of 2-dimensional echocardiography to measure right ventricular (RV) volume and ejection fraction was assessed in 22 children with congenital heart disease. From the apical 4 chambers 2-dimensional echocardiographic image, the long-axis length of the right ventricle was measured and the area planimetered. On the anteroposterior and lateral cineangiocardiographic planes, the right ventricle was separated into 2 parts: RV sinus and outflow tract. The longest length, inflow tract length, and area of the sinus were measured from biplane cineangiographic views. The echographic long-axis length correlated well with the longest length of the RV sinus measured from both anteroposterior and lateral cineangiographic views at both end-systole and end-diastole. Moreover, the echographic area correlated well with the sinus area obtained from both cineangiographic views. From these regression analyses, the echographic long axis length and area were corrected to the angiographic longest length and area of the sinus. The new corrected echographic longest length and area were applied to 3 formulas (2 biplane and 1 uniplane) to calculate the sinus volume of the right ventricle. Total RV volume was then derived from the sinus volume. RV volumes and ejection fraction determined by 2-dimensional echocardiography were compared with those obtained from biplane cineangiography using Simpsons rule method. All formulas tested predicted RV volumes and ejection fraction with equal accuracy. Thus, 2-dimensional echocardiography can assess RV volume and ejection fraction in children with congenital heart disease.

Accuracy of echocardiography in assessing left ventricular dimensions and volume.

SUMMARYThe accuracy of determining left ventricular function from echocardiography was assessed in 26 children (group I) with cineangiographically-determined normal left ventricular volume (LVV) and 28 children (group II) with large left ventricular volumes. Conventional LV echo dimensions were compared to the cineangiographic LV anterior-posterior minor axis (LVmA) and LVV. Very good correlations were found in group I between LV enddiastolic echo dimensions (LVEDD) and cine LVmA (r = 0.91) and between LVEDD and LV end-diastolic volume (LVEDV) by cine in group I (r = 0.86). In group II correlations were less accurate between LVEDD and diastolic LVmA and between LVEDD and LVEDV. There was poor correlation between the cine and echo percent of shortening (r = 0.41) and velocity of circumferential fiber shortening (VCF) (r = 0.51). This study demonstrates that M-mode echocardiography is a very useful method for determining LV dimensions in children with normal LV volume, but is less accurate in children with left ventricular volume overload or with abnormal septal orientation or postoperative status after ventriculotomy.

Modification of the Fontan Procedure Superior Vena Cava to Left Pulmonary Artery Connection and Inferior Vena Cava to Right Pulmonary Artery Connection With Adjustable Atrial Septal Defect

BACKGROUND A modification of the Fontan procedure with unidirectional cavopulmonary connection is described in which the superior vena cava (SVC) is connected to the left pulmonary artery (PA) and the inferior vena cava (IVC) is connected to the right PA via a lateral tunnel with a snare-controlled, adjustable atrial septal defect (ASD). This allows matching of the SVC and IVC flows with the lung of appropriate size. The obligatory left Glenn shunt provides an adequate arterial oxygen saturation, and the elevation in SVC pressure is well tolerated. The adjustable ASD allows selective decompression of the IVC that maintains cardiac output and reduces fluid accumulation in the serous cavities. METHODS AND RESULTS Since March 1992, we have performed this procedure in 18 patients. There were 17 children and 1 adult. Median age was 3 years and 9 months (range, 13 months to 36 years). Six patients had been staged with a previous bidirectional Glenn shunt. Preoperative cardiac catheterization revealed a PA pressure of 13 +/- 2 mm Hg and a transpulmonary gradient of 5 +/- 3 mm Hg. Ventricular function was satisfactory in all patients. At the completion of bypass, the pressures in the SVC and IVC were 16 +/- 4 mm Hg and 10 +/- 3 mm Hg, respectively (P < .01). The left atrial pressure was 6.0 +/- 3.0 mm Hg and the arterial O2 saturation on 100% oxygen was 93 +/- 3%. There was one death as a result of intractable atrial arrhythmias. The remaining 17 patients had a mean hospital stay of 9.7 days (6 to 18 days). The length of pleural drainage was 7 +/- 3 days. The ASD was adjusted in 11 patients before discharge. Oxygen saturation at discharge was 85.4 +/- 4%. Nine patients had repeat catheterization. The ASD was completely closed in 6 patients, an average of 2.5 months after surgery (range, 3 weeks to 5 months). After ASD closure, the arterial oxygen saturation was 96 +/- 3%, and the SVC and IVC pressures were both 13 +/- 3 mm Hg. CONCLUSIONS The Fontan procedure with unidirectional cavopulmonary connection and adjustable ASD has several advantages that may reduce mortality and morbidity for the high-risk Fontan candidate.

Two-dimensional echocardiographic assessment of left atrial size in children☆

The ability of 2-dimensional echocardiography (2-D echo) to estimate end-systolic left atrial (LA) size and volume was assessed in 140 infants and children. These subjects were divided into 2 groups. Group A included 91 patients with normal LA volume and Group B included 49 patients with LA volume overload. Five echocardiographic views (left parasternal long-axis, left parasternal short-axis, apical 4-chamber, apical 2-chamber and subcostal 4-chamber) were used. From these views, the LA long-axis and minor-axis lengths were measured and the area was planimetered. These echocardiographically derived measurements were compared with angiographically calculated LA volume. Although all echocardiographic measurements correlated well with angiographic LA volume measurements, the echocardiographic area tracked better than length measurements. Echo LA volume was calculated using 5 single-plane and 3 biplane area-length methods. LA volume calculated from either single- or biplane methods correlated well with angiographically determined LA volume. The degree of correlation depended on the method used. Echocardiographic area and estimated LA volume measured from the parasternal long-axis and apical 2-chamber views best separated patients with LA volume overload from normal. Two-dimensional echo using these views accurately segregated all patients with a LA volume greater than 180% of normal and 15 of 21 patients (71%) with an LA volume between 138% and 179% of normal. Thus, 2-D echo is useful in the evaluation of LA size and volume in infants and children.

Ebstein's malformation of the tricuspid valve with atresia: differentiation from isolated tricuspid atresia

A rare case of Ebsteins malformation of the tricuspid valve with atresia of the tricuspid orifice is presented. Although this case resembled tricuspid atresia in a physiologic or hemodynamic sense, embryologically and pathologically it should be grouped with Ebsteins anomaly of the tricuspid valve. Cases of the type presented are potentially correctable by surgery because of the anatomic features. It is therefore important to distinguish this lesion from isolated tricuspid atresia. Clinical findings, chest roentgenograms, electrocardiograms and vectorcardiograms may not be helpful in separating these two lesions. A right atrial angiogram outlined the obstructed and malformed tricuspid valve, which extended too far to the left and appears to be a distinguishing feature.