David E. Solowiejczyk

Two-dimensional echocardiographic assessment of right ventricular function as a predictor of outcome in hypoplastic left heart syndrome

This study was undertaken to assess the importance of right ventricular function at the time of initial presentation on early and intermediate outcome in patients with hypoplastic left heart syndrome (HLHS). Several studies have attempted to define physiologic risk factors for poor early outcome following the Norwood palliation for HLHS. No clinical or hemodynamic factors including right ventricular function have been found to reliably predict Norwood I operative survival. The relation between initial ventricular function and later survival has not been investigated. To assess the importance of right ventricular (RV) function at the time of initial presentation on outcome in patients with HLHS, systolic function was determined by qualitative and quantitative methods in 60 consecutive patients before surgical intervention. The effects on stage I operative survival, survival to stage II, and overall survival were analyzed. Initial RV function did not impact on stage I survival. However, analysis of later outcome of the stage I survivors showed that those with prestage I RV dysfunction had significantly greater mortality before stage II. Actuarial survival 18 months after Norwood surgery was 93% for patients with initially normal RV function compared with 47% for those with abnormal function (p = <0.005). The relative risk for later mortality was approximately 11 times greater for patients with initial RV dysfunction. Thus, RV dysfunction identifiable soon after initial presentation does not impact on early survival after Norwood I operation for HLHS. Intermediate and overall survival, however, is significantly decreased in patients with initially diminished RV function.

Ross Procedure in Infants and Toddlers Followed Into Childhood

Background—The Ross procedure is commonly used to treat aortic valve disease in pediatric and adult patients. For infants, data are limited regarding survival, reintervention, autograft growth, and function. Methods and Results—The Ross procedure was performed in 27 infants <18 months of age (median age 5.7 months). All patients had congenital aortic stenosis (AS); associated lesions included subAS (n=9), supravalvular AS (n=2), coarctation (n=5), and interrupted aortic arch (n=2). Median follow-up was 6.1 years (range 0.2 to 12.9). There were 3 early deaths and no late deaths. Freedom from reintervention for homograft dysfunction was 87% at 8 years; freedom from autograft reintervention was 100%. Follow-up echocardiograms were available in 17 patients. Estimated peak autograft gradient was 55 mm Hg in one patient and <10 mm Hg in 16. Mild autograft insufficiency was seen in 4 patients; 13 had none. Autograft diameter was measured early postoperatively and at latest follow-up. The mean z score increased from 0.63 to 3.2 (P<0.01) at the annulus and from 0.26 to 2.2 (P<0.01) at the sinus. In a subgroup, the mean autograft z score increased significantly from the postoperative period to 1 year for both the annulus (0.72 to 3.2, P<0.01) and the sinus (0.26 to 2.2, P<0.01), but remained unchanged thereafter. Conclusions—The Ross procedure effectively relieves AS in infants. Homograft reintervention occurred in 13% within 8 years. No patient developed significant autograft insufficiency or required autograft reintervention during the follow-up period. Dilatation of the autograft occurred during the first year after surgery and stabilized thereafter.

Pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: Case report and review of the literature

The pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect is highly variable. Several cases have been reported in the literature in which a coronary artery to pulmonary artery fistula, alone or in combination with additional vessels, supplies the pulmonary circulation. We report a case of successful repair during early infancy, with unique hemodynamic, clinical, and anatomic features. The literature is reviewed.

Bulboventricular Foramen Resection: Hemodynamic and Electrophysiologic Results

BACKGROUND The two major surgical approaches to the relief of bulboventricular foramen (BVF) obstruction in patients with single left ventricle (LV) are the Damus-Kaye-Stansel (DKS) procedure or direct BVF resection. Theoretical advantages of the DKS include better out-flow gradient relief, lower potential incidences of postoperative heart block and lower incidences of reoperation. Potential disadvantages of this approach include increased semilunar valvar insufficiency, lack of feasibility when attempting septation-type operations for univentricular hearts, and a technically more difficult operation. We report the results of direct surgical BVF resection. METHODS From June 1990 to June 1999, 9 patients had direct BVF resection performed at our institution. The median age at surgery was 16.5 years (range 1 month to 27 years). Diagnoses in these patients were [S,L,L] single LV (n = 8) and [S,D,D] single LV tricuspid atresia (n = 1). Eight of 9 patients had pulmonary artery bands placed either before BVF resection or at the same time as this procedure. Three patients required reoperation for reobstruction at the BVF (12 total operations in 9 patients). RESULTS Median preoperative peak systolic gradient across the BVF measured at cardiac catheterization was 47 mm Hg (range 10 to 63 mm Hg). The median peak gradient measured by Doppler echocardiography was 44 mm Hg (range 5 to 125 mm Hg). Eight of 9 patients survived the operation to discharge from the hospital and 7 of 9 are alive at follow-up. At a median follow-up of 22 months (range 5 to 76 months), 8 of 8 surviving patients had an unobstructed BVF as determined by qualitative two-dimensional echocardiography and Doppler color flow imaging. There was one perioperative and one late death 5 months postoperatively (secondary to fungal sepsis). No patient developed new or worsened aortic insufficiency after BVF resection. Eight of 9 patients had no change in AV nodal conduction after surgery. One patient developed Mobitz II heart block requiring postoperative implantation of a pacemaker. CONCLUSIONS Direct resection of an obstructive BVF can be performed with total relief of obstruction although reoperation may be required. Atrioventricular nodal function can be preserved in most patients with this operative approach, including those with [S,L,L] segmental anatomy.

Incidence of Aortic Root Dilatation in Pectus Excavatum and Its Association With Marfan Syndrome

OBJECTIVE To investigate the incidence of aortic root dilatation in pectus excavatum. DESIGN Retrospective medical record review and echocardiographic reanalysis. SETTING Morgan Stanley Childrens Hospital of New York-Presbyterian. PARTICIPANTS Surgical candidates with pectus excavatum (n = 37) and age-matched controls (n = 44) referred for an echocardiogram from 1994 to 2002. INTERVENTIONS Two-dimensional and color Doppler transthoracic echocardiograms. OUTCOME MEASURES The aortic annulus and root were measured and z scores were calculated and compared. Medical records were reviewed for genetic evaluation. RESULTS Patients with pectus excavatum and age-matched controls were reanalyzed. There was no difference in age, weight, height, or body surface area between patients and controls. There were no differences in the mean aortic annulus diameter, mean aortic annulus z score, or mean aortic root measurements. However, the aortic root z score was significantly higher in the pectus excavatum group compared with the controls: 0.9 (SD, 1.06) vs 0.0 (SD, 1.25) (P = .001). There were more patients with an aortic root z score of 2 or greater in the pectus excavatum group (9 of 37 patients) than in the control group (0 of 43 controls), with a calculated odds ratio of 29.7 (95% confidence interval, 1.10-1.59). Genetic evaluation was performed in 5 patients with a pectus excavatum and dilated aortic root; 2 of them received diagnoses of Marfan syndrome. CONCLUSIONS Aortic root dilatation is more common in patients with pectus excavatum than in a control population. Echocardiographic screening may be useful in the identification of aortic root dilatation in patients with isolated pectus excavatum.

Congenital Ductus Arteriosus Aneurysm Presenting With Stridor in a Newborn

A 2-day -old full term infant was found to have a weak cry and intermittent stridor, more so while agitated, both during inspiration and expiration. The history revealed an unremarkable pregnancy and normal spontaneous vaginal delivery. Mild respiratory distress requiring supplemental oxygen by nasal cannula was present. The heart sounds were best heard on the right hemi thorax with evidence of situs solitus. The lungs were clear and the chest radiograph is shown in Figure 1. A barium swallow study showed a lateral indentation in the upper esophagus at the level of the aortic arch. Laryngoscopy revealed left vocal cord palsy. Echocardiography showed a structurally normal heart with a left aortic arch and an abnormal suprasternal long axis view (Figure 2). A magnetic resonance imaging (MRI) study of the thorax was done (Figure 3). During hospitalization, the infant’s respiratory status deteriorated and required continuous positive airway pressure for 2 weeks. Serial echocardiograms were done and the infant was monitored closely for respiratory compromise. At 4 weeks, the infant became asymptomatic, baring occasional stridor, was weaned off from oxygen, started feeding by mouth, and gained weight. At 8 weeks of age, infant became totally free from stridor and a repeat chest Xray was done (Figure 4).

Simultaneous doppler and catheter transvalvular pressure gradients across st jude bileaflet mitral valve prosthesis: In vivo study in a chronic animal model with pediatric valve sizes

A mixture of valve types has been used in previous in vivo studies to assess the accuracy of Doppler echocardiography compared with catheter-measured pressure gradients across prosthetic mitral valves. However, limited data exist regarding the most commonly used bileaflet mechanical valve. We studied 14 sheep with St Jude Medical mechanical mitral valves. Continuous wave Doppler data were obtained across each of the 3 valve orifices. Hemodynamic data were obtained simultaneously by direct measurements with catheters. Valve sizes commonly used in the pediatric population in the mitral position (23 mm, 25 mm, and 27 mm) were studied. Linear regression analyses of Doppler-predicted versus catheter-measured gradients provided correlation coefficients ranging from 0.75 to 0.91. Agreement analysis demonstrated a scatter of Doppler data about the regression line. Although a reasonably good correlation of Doppler-predicted peak and mean pressure gradients across bileaflet mechanical valves exists in the mitral position, caution is needed when this method is applied to patients. Doppler overestimation was greatest across the 23-mm valves. Analyses of the specific orifice interrogated demonstrated higher estimated pressure gradients across the central orifice compared with the side orifices.