Improving reproducibility of left ventricular ejection fraction in pediatric oncology patients: less is more.

Reproducible measurement of left ventricular (LV) systolic function by echocardiography is important to detect cancer therapy-related cardiac dysfunction (CTRCD). We hypothesized that limiting the number of imaging operators and use of a single vendor would improve reproducibility of these measures. A standard operating procedure (SOP) whereby LV measurements were standardized and a cardio-oncology imaging team (5 sonographers, 6 cardiologists) was established. All pediatric oncology patient echocardiograms were acquired on a single vendor platform. In total, 100 consecutive pre-SOP and 100 post-SOP studies were reviewed. LV end-diastolic dimension (LVEDD), posterior wall thickness (PW), shortening fraction (SF), and ejection fraction by Simpson's biplane (EF) were re-measured by 2 blinded readers, and compared to what was originally reported. Image quality was scored by number of LV segments imaged (grades 1-4). Inter-observer reproducibility pre/post-SOP was assessed with intra-class coefficient (α). Reducing the number of imaging operators improved image quality (Grade ≥ 3: 13% vs. 46%, p < 0.001). Reproducibility of PW and LVEDD marginally improved (PW: 0.78 to 0.82; LVEDD: 0.96 to 0.97), and SF improved significantly (α = 0.65 vs. 0.79, p < 0.001). Pre-SOP reproducibility of LV EF was poor (α = 0.65), but improved significantly post-SOP (α = 0.83, p < 0.001). Reproducibility of LV EF improved with higher image quality score. Limiting imaging operators and vendor platform for pediatric oncology echocardiograms improves image quality and reproducibility of LV EF. Establishing an SOP and a cardio-oncology echocardiography team may improve precision of measurements used to detect CTRCD.

Safety and efficacy of intravenous contrast imaging in pediatric echocardiography.

This study was performed to determine the safety and efficacy of intravenous contrast echocardiography in children attending a tertiary cardiac center. This was a prospective study to evaluate the use of Optison contrast agent in children with severely limited transthoracic echocardiographic windows. Twenty children (median age, 15 years; range, 9-18) underwent fundamental imaging (FI), harmonic imaging (HI), and HI with intravenous contrast (Optison FS-069). Endocardial border delineation was determined based on a visual qualitative scoring system (0, none: 4, excellent). Endocardial border definition was significantly improved in all patients using contrast echocardiography (FI vs Optison, p < 0.001 for each). Improved border definition was most dramatic in the apical and left ventricular (LV) free wall regions. Left ventricular ejection fraction (LVEF) was measurable in 20 patients (100%) using contrast compared to 11 (55%) with FI or HI (p < 0.05). The echocardiographic diagnosis was correctly delineated in 1 patient with a severely dyskinetic LV segment only with use of intravenous contrast and HI. No patients suffered adverse hemodynamic effects, changes in taste, or flushing episodes. Three patients experienced transient headaches. Intravenous contrast echocardiography offers an additional tool in evaluating children with very poor transthoracic echocardiographic windows. Such a strategy increases diagnostic accuracy and allows accurate LVEF determination. Adverse hemodynamic effects related to intravenous contrast are exceedingly rare.

Patent foramen ovale morphology and impact on percutaneous device closure.

We sought to evaluate the effects of atrial septal and patent foramen ovale (PFO) morphology on the efficacy of transcatheter closure. We performed a retrospective analysis of all patients who underwent PFO device closure from January 1997 to January 2002. Forty-seven patients underwent percutaneous closure of a PFO with a median age of 45 years (range, 8-75) and weight of 76 kg (range 28-115). The septal morphology was flat in 33 and aneurysmal in 14 patients. The PFO morphology was a simple flap in 20 and complex in 27 patients. Complex morphologies included long-tunnel PFO (n = 15), coexistent small atrial septal defect (ASD) (n = 5), and aneurysmal septum without a tunnel or ASD (n = 7). Nonstretched PFO diameters were significantly smaller than stretched (4.8 +/- 1.1 mm vs 11.6 +/- 3.8 mm, p < 0.01). Median device size: stretched diameter ratio (DS:SD) was 3.7:1 (range, 2.2-9.1). The DS:SD ratio was significantly higher in patients with complex PFO (mean, 3.9:1 vs 2.6:1; p < 0.05). Device placement was successful in all patients. Five patients required transeptal puncture of the foraminal flap in long-tunnel PFOs. Effective closure on follow-up was achieved in 45 patients (95%). Of the 2 patients with residual shunts, 1 had a complex PFO and the other a simple PFO. Two patients (5%) experienced recurrent neurologic symptoms after device closure despite having no residual shunt by echocardiography. Complex PFO did not increase risk of residual shunt or recurrent neurologic symptoms after device closure.

Left ventricular noncompaction cardiomyopathy in association with trisomy 13.

In recent years, left ventricular noncompaction (LVNC) has been recognized as a distinct form of cardiomyopathy with its own clinical presentation and natural history. More than 100 cases of LVNC have been described in children. Although LVNC has been described in association with metabolic disorders such as Fabry's disease or genetic disorders such as Roifman's syndrome, this case represents the first report of LVNC in a child with trisomy 13.

Echocardiographic predictors of adverse clinical events in children with dilated cardiomyopathy: a prospective clinical study.

OBJECTIVES: To compare tissue Doppler (TD) velocities between patients with dilated cardiomyopathy (DCM) and normal controls and to determine whether TD velocities, Tei index, right ventricular fractional area change, and left ventricular ejection fraction (LVEF) predict adverse clinical outcomes in children with DCM. METHODS: Prospective evaluation of children with DCM. RESULTS: 54 children with DCM and 54 age and sex matched control group participants were studied. Mitral inflow velocities were similar for both groups except for decreased mitral deceleration time in patients with DCM. Systolic and diastolic TD velocities at the mitral annulus (septal and lateral sides) and tricuspid annulus were significantly reduced in children with DCM compared with controls (p < 0.001 for each). By multivariate analysis, after adjustment for Tei index and right ventricular fractional area change, decreased LVEF and tricuspid velocity during early diastole (Ea) were predictors of the primary end point (PEP), a composite end point consisting of need for hospitalisation or the outcome transplantation or death. Tricuspid Ea velocity < 8.5 cm/s had 87% specificity and 60% sensitivity for reaching the PEP. LVEF < 30% had 68% specificity and 74% sensitivity for the PEP. Combined LVEF < 30% and tricuspid Ea < 11.5 cm/s had 100% specificity and 44% sensitivity for the PEP. CONCLUSIONS: Children with DCM have significantly lower TD velocities than normal controls. In such cases, lower LVEF (< 30%) is more sensitive but less specific than lower tricuspid Ea velocities (< 8.5 cm/s) in predicting which patients are at risk of hospitalisation, transplantation, or death.

Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation.

The objectives of this study were to evaluate changes in dimension of the neo-aortic annulus, aortic root, and aortic anastomosis following arterial switch operation (ASO) and to identify risk factors for developing abnormal neo-aortic root enlargement and aortic regurgitation (AR). Prior studies report development of neo-aortic root dilatation and AR in a small subset of patients after ASO. Predisposing factors for neo-aortic root dilatation and development of moderate/severe AR are poorly understood. We performed a retrospective review of all patients with d-transposition of the great arteries (d-TGA) or double-outlet right ventricle with subpulmonary ventricular septal defect (VSD) who underwent ASO from May 1986 to January 2001. Serial echocardiograms were reviewed to measure neo-aortic annulus, root, and anastomosis diameter (z scores) and to determine progression of AR. Potential risk factors were assessed for developing neo-aortic root enlargement and AR. There were 119 patients (44 female and 75 male): 73 patients had simple d-TGA, 36 had d-TGA with ventricular septal defect, and 10 had a Taussig-Bing heart. The median duration of follow-up was 65 months (range, 12-180). The median neo-aortic root (z = 0.55+/-2.2; p < 0.01) and aortic annulus dimensions (z = 1.57+/-1.75; p < 0.01) were significantly increased over the study period. Aortic anastomosis diameter correlated with growth of the ascending aorta (z = 0.55+/-1.24). Development of severe neo-aortic root enlargement was associated with prior pulmonary artery (PA) banding (p < 0.01), the presence of a VSD (p = 0.03), and Taussig-Bing anatomy (p < 0.01) but was independent of coronary arterial anatomy, coronary arterial transfer technique, or associated lesions (p > 0.05). At latest follow-up, there was no or trivial AR in 88 patients, mild AR in 29 patients, and moderate to severe AR in 3 patients. Risk factors for developing mild or worse AR included severe or rapid neo-aortic root dilatation (p < 0.01). Only 3 patients required surgical intervention for AR. Despite the significant prevalence of neo-aortic root enlargement at intermediate follow-up after ASO, there is a low incidence of significant AR. Prior PA banding, the presence of VSD, and Taussig-Bing anatomy are risk factors for severe root enlargement. Surgical intervention for AR was rare (2%), however, serial surveillance of such patients is vital to monitor for neo-aortic root enlargement and potential aortic valve dysfunction.

Sizing of atrial septal defects to predict successful closure with transcatheter cardioSEAL device.

We conducted this retrospective study to compare methods for measuring atrial septal defects and to identify factors affecting echocardiographic measurement of such defects before transcatheter closure with the CardioSEAL'Septal Occluder. We reviewed the records of patients considered for device placement at our institution from January 1997 to April 1999. Atrial septal defect size was measured by transthoracic and transesophageal echocardiography; the stretched diameter was measured during catheterization by fluoroscopy and transesophageal echocardiography. The stretched-diameter fluoroscopic measurement was used for device size selection. Analysis of variance was used to calculate the effect of size, age, and size-by-age interaction. Thirty-one patients (3.3 to 72 years of age) underwent transthoracic and transesophageal echocardiography One patient was excluded from catheterization because of a 25-mm septal defect as indicated by transesophageal echocardiography (our maximum diameter, 15 mm). Thirty patients underwent transcatheter stretched-diameter sizing; 5 were excluded from device implantation because of defects >20 mm by stretched-diameter fluoroscopy (4) or septal length insufficient for device support (1). Implantation was successful in 23/25 patients; 2/23 had a residual shunt. In patients with available results (26/30), the stretched diameter was the same whether measured by stretched-diameter fluoroscopy or transesophageal echocardiography (P=0.007 R square=0.963). Compared with stretched-diameter fluoroscopy, precatheterization transthoracic and transesophageal echocardiography underestimated defect size by a mean of 22% and 13.2%, respectively. When data from those same tests were compared in defects of < or =0 mm and > 10 mm, transthoracic and transesophageal echocardiography were reliable predictors (P=0.003 and P=0.05, respectively) of stretched-diameter size in defects < or =0 mm.

Steerable control of the eustachian valve during transcatheter closure of secundum atrial septal defects.

Over the past decade there has been increased use of transcatheter devices for closure of secundum atrial septal defects. The presence of a large eustachian valve complicating transcatheter closure has not been described. We describe four patients with prominent eustachian valves, in three of whom we employed a simple technique to obtain control of the eustachian valve during device placement using transesophageal echo guidance.

Does ductal occlusion with the gianturco coil cause left pulmonary artery and/or descending aorta obstruction?

Thirty-two patients (median age 4.5 years) underwent transcatheter Gianturco coil occlusion of a patent ductus arteriosus. Transthoracic echocardiography was performed the day after coil placement and at intermediate follow-up (median 8.6 months). Echocardiographic results were compared with angiographic and hemodynamic data obtained during catheterization. Two-dimensional (2D) echocardiography performed the day after ductal occlusion displayed evidence of coil protrusion into the left pulmonary artery in 28 of 31 patients (90%) and into the descending aorta in 17 of 29 (59%). However, pulsed Doppler analysis demonstrated normal left pulmonary arterial flow velocities in 28 of 29 patients (97%) and normal descending aortic flow velocities in 26 of 27 (96%). Pulse Doppler results were corroborated by angiographic and hemodynamic catheterization data, which showed no evidence of adjacent vessel obstruction. Peak Doppler velocities among patients with and without 2D echocardiographic left pulmonary artery or descending aorta coil impingement did not differ significantly. The discrepancy between 2D and pulse Doppler findings did not change significantly at intermediate follow-up. Thus, transcatheter occlusion of the patent ductus arteriosus with properly implanted Gianturco coils does not cause significant obstruction to flow in the left pulmonary artery or descending aorta despite frequently misleading 2D echocardiographic images of coil impingement on these vessels.

Expression and secretion of beta-galactosidase in Saccharomyces cerevisiae using the signal sequences of GgpI, the major yeast glycosylphosphatidylinositol-containing protein.

New secretory signals and strategies can be attempted to improve the secretion of heterologous proteins of biotechnological interest which encounter difficulties being exported in yeast. The GGPI gene of Saccharomyces cerevisiae codes for a 125 kDa glycoprotein transported through the secretory pathway and anchored to the plasma membrane by means of a glycosylphosphatidylinositol. The regions coding for the secretory signal or also for the first 46 amino acids were tested for efficiency in secretion by fusion to the lacZ gene of Escherichia coli resulting in the synthesis of the endoplasmic reticulum-targeted 1-22- and 1-68-GgpIp/beta-gal hybrids. A cytoplasmic form was also examined. The 1-22 beta gal is partially transported to the cell surface and in the medium in an unglycosylated form. The 1-68 beta gal is completely retained in the intracellular membranes and is N-glycosylated in the GgpIp moiety. The amount of hybrid protein produced is similar and independent from its targeted site, suggesting that translocation through endoplasmic reticulum is not a limiting step, whereas the amount of active enzyme is from 50 to 80% lower for the endoplasmic reticulum forms compared with the cytoplasmic form. BiP/Kar2p putative precursor is accumulated in cells expressing the endoplasmic reticulum-targeted forms but not in those producing the cytosolic beta-galactosidase or over-expressing an endogenous secretory protein. Thus, glycosylation and abnormal folding rather than over-expression are among the factors responsible for the decreased activity and exit of beta-galactosidase from the endoplasmic reticulum and for induction of BiP. The results obtained indicate that the sole secretory signal of GgpIp is suitable to drive secretion of foreign products with complex folding and point to the importance of the endoplasmic reticulum quality control in the secretion of heterologous proteins in yeast.

Isolation of a novel beta4 integrin-binding protein (p27(BBP)) highly expressed in epithelial cells.

The integrin beta4 has a long cytodomain necessary for hemidesmosome formation. A yeast two-hybrid screen using beta4 cytodomain uncovered a protein called p27(BBP) that represents a beta4 interactor. Both in yeast and in vitro, p27(BBP) binds the two NH2-terminal fibronectin type III modules of beta4, a region required for signaling and hemidesmosome formation. Sequence analysis of p27(BBP) revealed that p27(BBP) was not previously known and has no homology with any isolated mammalian protein, but 85% identical to a yeast gene product of unknown function. Expression studies by Northern analysis and in situ hybridization showed that, in vivo, p27(BBP) mRNA is highly expressed in epithelia and proliferating embryonic epithelial cells. An antibody raised against p27(BBP) COOH-terminal domain showed that all beta4-containing epithelial cell lines expressed p27(BBP). The p27(BBP) protein is insoluble and present in the intermediate filament pool. Furthermore, subcellular fractionation indicated the presence of p27(BBP) both in the cytoplasm and in the nucleus. Confocal analysis of cultured cells showed that part of p27(BBP) immunoreactivity was both nuclear and in the membrane closely apposed to beta4. These results suggest that the p27(BBP) is an in vivo interactor of beta4, possibly linking beta4 to the intermediate filament cytoskeleton.

Update on new technologies in pediatric echocardiography.

Advances in ultrasound technology will continue to expand the utility of echocardiography in the assessment of structural and functional cardiac disease in children. Tissue Doppler imaging and dobutamine stress echocardiography are 2 promising clinical applications that are expected to become increasingly used with time. Advances in data compression technology, including JPEG and MPEG techniques, will significantly affect digital archival and transmission of echocardiograms, which also have clinical implications, particularly in the expanding use of telemedicine. Continued research and clinical experience will further define the ultimate roles of these technologies in the future.

Gene mapping of familial autosomal dominant dilated cardiomyopathy to chromosome 10q21-23.

Dilated cardiomyopathy (DCM) is the most common form of primary myocardial disorder, accounting for 60% of all cardiomyopathies. In 20-30% of cases, familial inheritance can be demonstrated; an autosomal dominant transmission is the usual type of inheritance pattern identified. Previously, genetic heterogeneity was demonstrated in familial autosomal dominant dilated cardiomyopathy (FDCM). Gene localization to chromosome 1 (1p1-1q1 and 1q32), chromosome 3 (3p25-3p22), and chromosome 9 (9q13-9q22) has recently been identified. We report one family with 26 members (12 affected) with familial autosomal dominant dilated cardiomyopathy in which linkage to chromosome 10 at the 10q21-q23 locus is identified. Using short tandem repeat polymorphism (STR) markers with heterozygosity > 70%, 169 markers (50% of the genome) were used before linkage was found to markers D10S605 and D10S201 with a pairwise LOD score = 3.91, theta = 0, penetrance = 100% for both markers. Linkage to 1p1-1q1, 1q32, 3p25-3p22, and 9q13-9q22 was excluded. We conclude that a new locus for pure autosomal dominant FDCM exists, and that this gene is localized to a 9 cM region of 10q21-10q23. The search for the disease causing gene and the responsible mutation(s) is ongoing.

Genetic heterogeneity in familial dilated cardiomyopathy.

Familial dilated cardiomyopathy (FDCM), an inherited primary form of myocardial disease, is a significant cause of morbidity and mortality at all ages and the leading reason for cardiac transplantation worldwide. Although typically inherited as an autosomal dominant disorder, all forms of inheritance have been recognized. FDCM appears to be responsible for approximately 20-30% of all cases of dilated cardiomyopathy, the most common form of cardiomyopathy. Recently, two families having autosomal dominant FDCM were mapped. The first family had conduction abnormalities and FDCM and was mapped to 1p1-1q1, while the second family, which had pure FDCM, was mapped to 9q13-q22. Neither gene has been identified to date. In this report, one family with pure FDCM was analyzed for linkage to the 1p1-1q1 and 9q13-q22 loci using parameteric linkage analysis, with linkage to both regions excluded. This demonstrates that the pure form of FDCM is caused by multiple different genes, i.e., genetic heterogeneity. Identification of large families with FDCM will be required to identify the various genes responsible for this important clinical entity.

Quantitative morphometric analysis of progressive infundibular obstruction in tetralogy of Fallot. A prospective longitudinal echocardiographic study.

BACKGROUND: The morphological hallmark of tetralogy of Fallot is controversial, with much disagreement as to whether the subpulmonary infundibulum in this lesion is hypoplastic. In addition, few quantitative data are available regarding the morphometry of the subpulmonary infundibulum, what anatomic characteristics are acquired in the postnatal period, and at what rate they progress. We also sought to determine whether echocardiographic morphometric analysis of the infundibulum can predict clinical course in infants with tetralogy of Fallot. METHODS AND RESULTS: Twenty-one infants with tetralogy of Fallot (median age at initial study, 1.6 months) were prospectively followed with serial echocardiograms until the time of first surgical intervention (median age at surgery, 10 months). Selected video still frames were digitized off-line with a computerized system. Compared with age-matched normal control infants (n = 37), the following indexed infundibular dimensions in patients with tetralogy of Fallot were significantly smaller: length (1.86 +/- 0.54 versus 2.7 +/- 0.56 cm/BSA0.5, P < .0001), cross-sectional area (1.6 +/- 0.49 versus 4.7 +/- 1.3 cm2/BSA, P < .0001), and volume (1.24 +/- 0.62 versus 7.2 +/- 3 mL/BSA1.5, P < .0001). The following measurements were increased in tetralogy patients: infundibular septal thickness (0.83 +/- 0.21 versus 0.54 +/- 0.06 cm/BSA0.5, P = .0002) and infundibular free-wall thickness (0.62 +/- 0.13 versus 0.49 +/- 0.06 cm/BSA0.5, P = .006). The angle between infundibular septum and ventricular septum had a greater degree of anterosuperior deviation in tetralogy patients, resulting in a larger infundibuloventricular septal angle (77 +/- 8.2 degrees versus 31 +/- 6.5 degrees, P < .0001). During follow-up, infundibular volume in tetralogy patients decreased from 1.24 +/- 0.62 to 0.81 +/- 0.47 mL/BSA1.5 (P = .002), correlating with infundibular septal thickness (r = -.63, P < .003). The mean rate of decrease of indexed infundibular volume was 0.1 +/- 0.13 mL.BSA-15.mo-1. Correlation analysis revealed a nonlinear correlation between the degree of infundibular septal malalignment and indexed infundibular volume (r = .93, P < .0001). Tetralogy patients who required early surgical intervention (4.8 +/- 0.9 versus 10.7 +/- 1.7 months, P < .0001) had a smaller infundibulum at presentation (0.92 +/- 0.35 versus 1.41 +/- 0.67 mL/BSA1.5, P = .04) and an accelerated rate of infundibular narrowing (0.17 +/- 0.18 versus 0.06 +/- 0.08 mL.BSA-1.5.mo-1, P = .04). CONCLUSIONS: Compared with normal infants, the subpulmonary infundibulum in tetralogy of Fallot is characterized by a smaller volume, shorter and thicker infundibular septum, and anterosuperior deviation of the infundibular septum. Infundibular obstruction in tetralogy patients is progressive, with an average rate of decrease in indexed infundibular volume of 0.1 +/- 0.13 mL.BSA-1.5.mo-1. Infants who are likely to require early therapeutic intervention may be identified on their initial echocardiogram as having an infundibular volume of < 0.9 to 1.0 mL/BSA1.5.

Quantitated left ventricular systolic mechanics in children with septic shock utilizing noninvasive wall-stress analysis.

OBJECTIVE: To quantitate ventricular systolic mechanics in septic children. DESIGN: Prospective wall-stress analysis was compared to standard ejection phase indices. SETTING: University-based pediatric intensive care unit. PATIENTS: Fifteen children with sepsis (hemodynamically stable, n = 5; in shock, n = 10). MEASUREMENTS AND MAIN RESULTS: Left ventricular ejection phase indices: shortening fraction (shortening) and corrected mean velocity of circumferential shortening (velocity) were adjusted for end-systolic wall stress (stress). Ejection phase, performance (stress-shortening relation), contractility (stress-velocity relation), and afterload (stress) were indexed to age-corrected normal means, with variance of > or = 2 SD regarded as significant. Preload index represented variance between performance and contractility indices. All hemodynamically stable septic patients had normal performance, contractility, and preload. Afterload was increased in three of five patients. Of the patients with septic shock, six of ten had decreased performance (decreased contractility and increased afterload, n = 4; decreased afterload, n = 1; and severe preload deficit, n = 1). Despite aggressive volume resuscitation, six of ten children in septic shock had evidence of diminished preload. Follow-up studies in the septic shock patients demonstrated reversal of depressed ventricular contractility within 3 to 6 days in all four patients initially affected (p < .05). One patient developed late decreased performance and contractility in association with multiple organ failure. Ventricular loading abnormalities persisted in a follow-up study of these patients including a preload deficit in five of ten patients in shock. CONCLUSIONS: The frequency rate (40%) of reversible impaired ventricular contractility in children with septic shock is significant. Afterload is normal or increased in the majority of septic subjects, possibly due to acute ventricular dilation. Decreased preload contributes to altered ventricular performance in the majority of children with septic shock, persisting days after the initiation of therapy. Wall-stress analysis provided detailed information regarding ventricular mechanics that was not otherwise obtainable by standard ejection phase indices.

[Emergency treatment of ureteral colic with nifedipine]. / Trattamento in urgenza delle coliche ureterali con nifedipina.

The Authors evaluated clinical efficacy of nifedipine versus cymetropio bromide in relieving acute ureteral colic in 40 patients. Rapidity, efficacy and time of these drugs' activity in relieving pain of ureteral colic has been evaluated. The investigation shows as nifedipine relieves acute ureteral pain more quickly than cymetropio bromide (respectively 5 minutes in 95% of patients, 20 minutes in 65% of patients), but for a brief time (pain recurrence respectively in 70% and 25% of patients).

[Aortic coarctation and associated abnormalities in the first year of life. Echocardiographic diagnosis]. / Coartazione aortica e anomalie associate nel primo anno di vita. Diagnosi ecocardiografica.

Thirty-eight cases of "Aortic Coarctation Syndrome" presenting in the first year of life (66% under 3 months of age) were studied with cross-sectional echocardiography. Direct imaging of the coarctation was achieved in 75% of cases. Patent ductus arteriosus was present in 60% (80% before three months). Associated anomalies were present in 63%; VSD 29%, Aortic stenosis 16%, Mitral stenosis 16%, AV Canal 5%, Taussig Bing type of DORV 5%, Corrected transposition with VSD 3%, Ebstein anomaly 3%, Univentricular A-V connection 3%. The results were compared with angiographic and/or surgical and/or autoptic findings. The echocardiographic diagnosis proved to be very reliable in most cases. The policy of sending to surgery most neonates and infants with coarctation of the aorta without preoperative catheterization is discussed.