Natural history and prognostic risk factors in endocardial fibroelastosis.

A retrospective study was made of 52 patients (16 men, 36 women) with endocardial fibroelastosis diagnosed by strict clinical criteria and confirmed histologically in 18 (35%). Clinical and hemodynamic manifestations at presentation were reviewed from the clinical record. The follow-up period averaged 47 months (range 1 day to 228 months).

Closed heart surgery for congenital heart disease in infancy.

Closed heart surgery in congenital heart disease can be palliative or corrective. Palliative surgery aims at improving the physiologic deficit rather than the anatomic defect of the heart. Palliative procedures aim to increase pulmonary blood flow in cyanotic children with decreased pulmonary blood flow (Blalock-Taussig shunt), decrease pulmonary blood flow when there is unrestricted flow (pulmonary artery banding), or improve venous mixing in cyanotic children that require pulmonary and systemic venous mixing for survival (atrial septectomy).

Left ventricular systolic and diastolic function after two-stage anatomic correction of transposition of the great arteries.

Anatomic repair of transposition of the great arteries (TGA) has been developed because of concerns about right ventricular function after atrial repair by the Mustard or the Senning technique. This study assessed left ventricular systolic and diastolic function in three patients after two-stage anatomic repair. Two patients had a ventricular septal defect (one with coarctation), and the third patient had right ventricular dysfunction precluding atrial repair.

Cardiac manifestations in disorders of fat and carnitine metabolism in infancy.

The prognosis of patients with cardiomyopathy associated with hypocarnitinemia is uncertain. Cardiac hemodynamics, histologic findings and response to oral L-carnitine therapy were retrospectively evaluated in 11 children with cardiomyopathy associated with abnormal carnitine metabolism. Three had systemic carnitine deficiency, two familial hypocarnitinemia with neutropenia, three transient neonatal hypocarnitinemia and three a carnitine insufficiency syndrome.

Cardiopulmonary adaptation at rest and during exercise 10 years after Mustard atrial repair for transposition of the great arteries.

Discordance exists between apparently reduced systemic right ventricular function and the reported asymptomatic state of many patients after atrial repair for transposition of the great arteries. To evaluate this clinical observation, cardiopulmonary response to exercise in 17 asymptomatic patients with no significant postoperative hemodynamic abnormalities was assessed by upright bicycle ergometry according to a modified Jomes protocol 11.5 +/- 1.

Repair of tricuspid atresia: utility of right ventricular incorporation.

During a 10-year period, 62 patients underwent the following modifications of the Fontan operation for repair of tricuspid atresia: direct atriopulmonary connection (N = 15), atriopulmonary connection using a conduit (N = 5), direct atrioventricular (AV) connection (N = 22), and AV connections with a valved conduit (N = 20), including 2 with combined Fontan-arterial switch procedures. The overall hospital mortality was 16.1% (10/62) (70% confidence limits, 11.

An echocardiographic study of the association of ventricular septal defect and right ventricular muscle bundles with a fixed subaortic abnormality.

Since 1983, 36 patients with the combination of right ventricular muscle bundles and a perimembranous ventricular septal defect have been studied in our institution to address the incidence of on associated subaortic abnormality. Of that total 32 (88%) had echocardiographic evidence of such an abnormality (29 had a typical subarotic ridge protruding from the crest of the interventricular septum and the remaining 3 had an echodense area in the same location). Surgical confirmation of the presence or absence of a subaortic abnormality was available in 26.

Early and late results of a protocol for simple transposition leading to an atrial switch (Mustard) repair.

Inferences: A treatment protocol for patients with simple transposition, consisting of initial balloon atrial septostomy and leading to a delayed atrial switch repair of the Mustard type, in good circumstances, results in survivorship at 1 month, 1 year, 10 years, and 20 years of 95% (95% confidence limits [CL] 90% to 97%), 90% (95% CL 84% to 94%), 83% (95% CL 74% to 90%), and 80% (95% CL 70% to 88%), respectively. Low birth weight adversely affects survivorship. Hypoxia or heart failure accounts for most deaths within the first month and before the atrial switch (Mustard) repair.

Angiographic features associated with percutaneous balloon valvotomy for pulmonary valve stenosis.

Changes in the mobility of the pulmonary valve were determined by a retrospective review of right ventricular cineangiograms from 25 balloon pulmonary valvotomy (BV) procedures in 23 infants and children. The angiographic changes were compared with the post-BV catheter and Doppler pressure gradients across the right ventricular outflow tract. Angiographic features felt to indicate valve tearing were present following 17 of 25 procedures and included increased excursion or straightening of leaflets, localized change in leaflet motion (flail leaflet), and the presence of an additional contrast jet through the valve.

Thrombolytic therapy for femoral artery thrombosis after pediatric cardiac catheterization.

Femoral artery thrombosis remains a well-known complication after cardiac catheterization. A study was undertaken to assess the efficacy of thrombolytic therapy for this complication. A total of 526 consecutive infants and children were prospectively evaluated after cardiac catheterization, and the medical charts of 42 patients who required femoral artery thrombectomy between 1975 and 1985 were reviewed.

Correlation of left ventricular angiographic casts and biplane left ventricular volumetry in infants and children.

To calculate left ventricular (LV) volumes from biplane cineangiography obtained in nonstandard views, regression equations were developed from LV casts of known volume. Volumes were calculated by the area-length method from casts ranging from 1.4 to 48.

Correlation of right ventricular volume using axial angulated ventriculography to known right ventricular cast volumes in infants and children with congenital heart disease.

To calculate right ventricular (RV) volumes from biplane cineangiography obtained in nonstandard views, regression equations were developed from RV casts of known volume. Volumes were calculated using Simpson's rule from casts ranging from 2 to 42 ml from 25 postmortem specimens with various congenital heart diseases. The casts were divided into 2 groups: group 1 (n = 15) with abnormal or group 2 (n = 10) with normal RV hemodynamic measurements.

Validation of Doppler-derived pulmonary arterial pressure in patients with ductus arteriosus under different hemodynamic states.

Twenty-nine patients with a patent ductus arteriosus (PDA) in isolation (n = 17) or in combination with other lesions (n = 12) underwent simultaneous hemodynamic assessment and evaluation of PDA flow velocity by the Doppler method. The accuracy with which Doppler velocity across the PDA predicted pulmonary arterial pressure and the influence of PDA size and shape on the Doppler velocity-pressure relationship were examined. Seventy percent had a cone-shaped PDA (narrowest at the pulmonary artery end), and the remainder were tubular.

Critical aortic stenosis. Survival and management.

The factors associated with survival in 40 neonates (age less than 28 days) with critical aortic stenosis undergoing either open (22 patients) or closed (18 patients) transventricular aortic valvotomy were reviewed. Significant adverse correlates with survival included evidence of poor perfusion preoperatively (low pH, greater than Grade 2/6 soft ejection systolic murmur) and marked congestive heart failure (hepatomegaly, cardiomegaly, elevated left atrial pressure). Congenital mitral stenosis (anulus less than 11 mm), a small aortic anulus (less than 6.

The morphology of the right ventricular outflow tract after percutaneous pulmonary valvotomy: long term follow up.

Twenty nine patients (19 male, mean (SD) age 6.25 (0.5) years (range 0.

Results with the Mustard operation in simple transposition of the great arteries 1963-1985.

From May 1963 to December 1985, 329 infants and children underwent repair of simple transposition of the great arteries (TGA) using Mustard's technique. To assess improvement, the patients were separated into two groups by date of operation: Group I, May 1963 to December 1973 (N = 106), and Group II, January 1974 to December 1985 (N = 223). The operative mortality was 11 (10.

Surgical experience with reparative techniques in patients with congenital mitral valvular anomalies.

Our review of an entire institutional experience with primary repair of congenital and acquired mitral valve (MV) anomalies in children with concordant atrial-ventricular-arterial connections but without atrioventricular septal defects included 48 patients (1962 to September 1986). Mitral stenosis (MS) was the predominate lesion in 24 patients, mitral incompetence in 22, and mixed in two. Most patients with MS had so-called typical congenital MS (Van Praagh) with abnormalities of all valvular components; virtual or complete absence of chordal development was a consistent finding.

Surgical experience with the modified Fontan procedure.

During a 10 year period 109 patients (3 months to 47 years old) underwent modifications of the Fontan procedure for repair of classic tricuspid atresia (TA) (n = 58), univentricular atrioventricular connection (UVH) (n = 38), or other complex malformations (CM) (n = 13). Among patients with TA, an atriopulmonary connection was used in 19 (33%) and incorporation of the right ventricle with the Björk modification and with a right atrial-to-right ventricular valved conduit was used in 20 (34%) and in 19 (33%), respectively. Three of the latter 19 also underwent a combined Fontan-switch procedure.

Tricuspid valve replacement in children.

Between 1974 and January, 1986, 11 children underwent 13 tricuspid valve replacements at the Hospital for Sick Children in Toronto. Age at operation ranged from 24 hours to 14.5 years (mean, 6.

Two-dimensional and pulsed Doppler echocardiography in the postoperative evaluation of total anomalous pulmonary venous connection.

The role of combined two-dimensional and pulsed Doppler echocardiography in the postoperative assessment of patients with total anomalous pulmonary venous connection was evaluated. Twenty-two cases with a median age of 9.5 weeks at the initial examination were evaluated.

The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation.

The feasibility of using balloon dilatation to relieve stenosis caused by dysplasia of the pulmonary valve was assessed in seven patients (five female, mean age two years) with angiographically confirmed dysplasia who were identified among 38 patients with pulmonary valve stenosis selected for balloon dilatation over a two year period. The clinical features in three patients were consistent with Noonan's syndrome. In all patients the gradient across the valve was assessed by cross sectional echocardiography and Doppler echocardiography before cardiac catheterisation.