Double-inlet single left ventricle: Echocardiographic anatomy with emphasis on the morphology of the atrioventricular valves and ventricular septal defect

AbstractThe echocardiographic anatomy of double inlet single left ventricle was studied in 57 patients, aged 1 day to 27 years (mean 6 years); the variables examined included morphology, size and function of the atrioventricular AV) valves and ventricular septal defect and their relation to pulmonary stenosis, aortic stenosis and aortic arch obstruction. The visceroatrial situs was solitus and the heart was in the left side of the chest in all 57 patients. A d-loop ventricle was present in 21 patients and an l-loop ventricle in 36. The great arteries were normally related (Holmes heart) in 8 patients and transposed in 49.In all hearts, the right AV valve was anterior to the left AV valve. In 53 patients, the tricuspid valve (right valve in d-loop and left valve in l-loop) was closer to and had attachments on the septum. The tricuspid valve straddled the outflow chamber in eight patients. No significant difference was noted in the mean AV valve diameter when comparing mitral and tricuspid valves within the same group or between the groups with a d- or l-loop ventricle. The right AV valve diameter had a significant direct correlation with the aortic valve diameter and the size or (he ventricular septal defect regardless of ventricular loop. Both AV valves were functionally normal in 34 patients. Among patients with AV valve dysfunction, the tricuspid valve tended to be stenotic in patients with an l-loop ventricle and regurgitant in patients with a d-loop ventricle. Mitral valve dysfunction was uncommon.The ventricular septal defect (46 patients) was separated from the semilunar valves in 24 patients (muscular defect) and adjacent to the anterior semilunar valve as a result of hypoplasia or malalignment, or both, of the infundibular septum (subaortic defect) in 19 patients. Multiple defects were present in three patients. The difect was unrestrictive in 26 patients, restrictive in 23 and could not be evaluated in 8. Pulmonary artery banding had been performed in 8 of the 26 patients with an unrestrictive defect and in 10 of the 23 patients with a restrictive defect. Only 4 of 19 subaortic defects compared with 16 of 24 muscular defects were restrictive. The size of the defect was significantly correlated with the measured pressure gradient. Among patients with transposition, only 2 of 13 with pulmonary stenosis had a restrictive ventricular septal defect compared with 15 of 30 without pulmonary stenosis. In patients with transposition, the defect size was significantly smaller when coarctation was present

Transcatheter closure of a large patent ductus arteriosus with the clamshell septal umbrella

AbstractIn 14 patients undergoing transcatheter closure of a large (>4 mm diameter) patent ductus arteriosus, occlusion was attempted with use of the Bard Clamshell septal umbrella. Patient age ranged from 0.7 to 30.4 years. Isolated patent ductus arteriosus was present in 11 patients; 3 had additional congenital heart lesions. Moderate or severe pulmonary hypertension was present in four patients. The diameter of the patent ductus arteriosus ranged from 4.5 to 14 mm, as determined by contrast injection through an 11F sheath or by balloon sizing; it appeared larger by this method than by the standard angiographic method.All 14 patent ductus arteriosi were successfully closed. Prior embolization of a Rashkind umbrella was the reason for using a Clamshell device in three patients; one additional embolization of a Clamshell device occurred. All errant devices were retrieved at cardiac catheterization, without associated hemodynamic instability. No other complications occurred. Among the 14 patients, 11 had complete ductal closure by Doppler color flow mapping at last follow-up and 3 had trivial residual Row. All four patients having associated complex lesions or pulmonary hypertension, or both, had symptomatic improvement after the procedure, although one child (with Shone's anomaly) died 3 months later.The Clamshell device provides stable and effective closure of a large patent ductus arteriosus, and allows transcatheter closure to be offered to some patients who were previously considered unsuitable for this procedure

Obstruction of right ventricular inflow and outflow in corrected transposition of the great arteries {S,L,L}: Two-dimensional echocardiographic diagnosis

Obstruction of systemic ventricular inflow and outflow is considered uncommon in corrected transposition of the great arteries {S,L,L}. Between 1979 and 1985, 42 patients with corrected transposition and two ventricles and atrioventricular valves underwent two-dimensional echocardiography. Obstruction of right ventricular inflow and outflow was present and diagnosed by two-dimensional echocardiography in 5 of the 42 patients. A supratricuspid stenosing ring, recognized in the apical or subxiphoid four chamber view as a bright, linear structure on the left atrial side of the tricuspid valve, occcurred in two patients. Subaortic obstruction due to infundibular hypertrophy with or without displaced muscle bundles was seen in three patients. Subxiphoid long- and short-axis views and parasternal long-axis views best displayed these features. Aortic coarctation was present in four cases and could be diagnosed using modified suprasternal notch views.Thus, systemic ventricular inflow and outflow obstruction may be more common in corrected transposition than previously believed (occurring in up to 10 to 15% of patients). The mechanisms producing the obstruction appear to be characteristic of the left atrium and right ventricle irrespective of location or connections. Echocardiography appears to be an excellent technique for diagnosing these associated lesions in corrected transposition

Truncus Arteriosus and Absent Ascending Aorta With Unusual Head and Neck Vessel Origins

We describe a neonate with a unique variant of truncus arteriosus with interrupted aortic arch, an absent ascending aorta, persistent right dorsal aorta, and an unusual brachiocephalic artery pattern in which all head and neck vessels were supplied from the ductal arch-descending aorta continuum. (Level of Difficulty: Beginner.

Correlation of transthoracic echocardiography-derived pulmonary to systemic flow ratio with hemodynamically estimated left to right shunt in atrial septal defects

Background : Transthoracic echocardiographic (TTE) estimation of the pulmonary to systemic flow ratio (Qp/Qs) is routinely used in clinical practice and is included in the American Society of Echocardiography Guidelines. We sought to assess its real-world applicability with a particular focus on hemodynamically significant shunt lesions. Methods : Retrospective single institutional review of TTE's in patients with secundum atrial septal defect prior to cardiac catheterization (cath) from 2012 to 2018 was performed (n = 109), those with technically limited images for Qp/Qs calculation (n = 11) and those with time interval between TTE and cath >60 days were excluded (n = 14). Qp/Qs was calculated from stored clips by previously described methods and correlated with those obtained by oximetry. Patients were subdivided into two age groups <21 (Group 1) and ≥22 years (Group 2). TTE and cath methods for Qp/Qs estimation were compared using paired t-test, Pearson's correlation coefficient, and Bland–Altman plots. Results : Eighty-four subjects met inclusion criteria (age range 3–78 years). Group 1 n = 35; median age 10 years; Group 2 n = 49; median age 49 years. Transthoracic echocardiogram was performed 19.5 ± 15 days prior to cath. Mean Qp/Qs derived by cath and TTE were 2.09 ± 0.9 versus 2.54 ± 1.2 (P < 0.0001). Overall correlation was poor between the methods (r2 = 0.32, P < 0.0001) and continued to be poor for Groups 1 and 2 (r2 = 0.24, P = 0.003 and r2 = 0.40, P < 0.0001 respectively). Bland–Altman plots demonstrated poor agreement between the predetermined limits of agreement (-0.5–1.5). Conclusion : Transthoracic echocardiography estimated Qp/Qs, although routinely utilized in clinical practice, has poor correlation and agreement with oximetry-derived Qp/Qs. The test performs poorly in all age groups in detecting a hemodynamically significant shunt and tends to overestimate the degree of left to right shunt

A comparison of echocardiographic techniques in determination of arterial elasticity in the pediatric population

BACKGROUND: Many methods are used to measure arterial elasticity in children using echocardiography. There is no data to support the equivalence of the different techniques. The goal of this study was to evaluate the reproducibility of several techniques used to measure arterial elasticity using echocardiography. METHODS: Aortic distension in two different sites (arterial distension) through the cardiac cycle was measured by (four) two-dimensional (2D) and M-mode echocardiographic techniques in 20 children without significant structural heart disease. These measurements combined with noninvasive blood pressure measurements were used to calculate arterial elastic indices. Arterial elasticity was expressed in terms of distensibility and stiffness. Data were collected by two sonographers and interpreted by two reviewers. Paired Student\u27s t-test and Pitman\u27s test for equality of variance for correlated observations were used to detect differences between different sonographers, different reviewers, and different techniques. RESULTS: No significant difference in the measured elasticity between sonographers or reviewers was observed. There was a somewhat increased variance in two of the four techniques evaluated. There was no significant difference in elasticity measured using different techniques to evaluate the same arterial site, although a significantly decreased elasticity was noted from measurements taken in the proximal ascending aorta as compared with the distal ascending aorta. CONCLUSIONS: Many echocardiographic techniques produce reproducible measurements of arterial elasticity. There may be intrinsic differences in arterial elasticity between different segments of the ascending aorta, which have not been previously described in children with normal cardiac anatomy. Comparisons of data from separate studies must take these differences into account