Frank M. Galioto

Left ventricular false tendons in children: Prevalence as detected by 2-dimensional echocardiography and clinical significance

During a 28-month period, consecutive 2-dimensional echocardiograms were reviewed to determine the prevalence of left ventricular (LV) false tendons, their associated anomalies and clinical significance. LV false tendons were found in 31 (0.8%) of 3,847 consecutive 2-dimensional echocardiograms. Of 31 LV false tendons, 30 passed longitudinally from papillary muscle to septum and 1 went from free wall to free wall. The 31 patients were aged 1 day to 15 years. Associated heart disease, most often ventricular septal defect, bicuspid aortic valve and coarctation of the aorta, was present in 48%, of whom 73% were girls. Of those without heart disease, 69% were boys. In patients with heart disease, precordial murmurs were due to the underlying cardiac anomaly. Of those without heart disease, 15 of 16 (94%) had a precordial murmur, usually of the Stills type over the lower left sternal border. Four of 31 (13%), 1 with and 3 without heart disease, had unifocal premature ventricular contractions that were rate-dependent in the 2 patients undergoing stress testing. LV false tendons appear to occur in 0.8% of pediatric patients and usually are accompanied by a Stills type innocent murmur if unassociated with heart disease. Some LV false tendons are associated with rate-dependent premature ventricular contractions.

Small left atrium and change in contour of the ventricular septum in total anomalous pulmonary venous connection: A morphometric analysis of 22 infant hearts

Morphometric measurements of 22 hearts with total anomalous pulmonary venous connection (TAPVC) were compared with measurements of 8 matched control specimens without heart disease. Each of the TAPVC specimens had a shorter left atrium, smaller left atrial surface area and larger diameter of the fossa ovalis. In addition to increased length of the right ventricle and larger circumferences for tricuspid and pulmonary valve anuli, the left ventricular contour of the ventricular septum was flat or convex in 18 of the 22 hearts; the septum was significantly longer than normal in these specimens and wider at its midportion. Because mitral and aortic valve anuli were normal in circumference, the data suggest that left ventricular volume is not decreased despite change in ventricular shape.

Measurement of tracheal airflow in newborns by a differential flow system.

The differential arrangement of two pneumotachographs in a gas blow-by system, upstream and downstream from the patient connection port, permits accurate measurement of the tracheal air flow without introduction of flowmeter dead-space. The method does not require but will accept a constant background gas flow rate and permits humidification and easy switching of the inhalant gases. The admissible background flow rates limiting measurement errors below 5% in the range of tidal volumes relevant for newborns for two sizes of commercially available pneumotachographs were found to be -3 L/min and 0-12 L/min, respectively. The lack of restrictions imposed on the properties of the background flow source makes the differential system especially suitable for measurement of ventilation and pulmonary mechanics in spontaneously breathing or mechanically ventilated newborn infants.

Single ventricle with truncus arteriosus in siblings

Abstract Single ventricle and truncus arteriosus (TA) are relatively uncommon congenital cardiac defects. Keith et al. 1.2 reported these lesions to occur, respectively, in 1.1% and 0.4% of children with congenital cardiac defects. The occurrence of TA with a single left ventricle (LV) often has been theorized as a developmental stage but not previously described. This report describes siblings with the unique association of single LV with rudimentary right ventricular (RV) outflow chamber, TA type I, ostium primum atrial septal defect (ASD), and common atrioventricular (AV) valve.

Experience with Repair of Complete Atrioventricular Canal

Twenty-nine patients at out institution have undergone repair of complete atrioventricular canal since 1969. There were 3 operative and 5 late deaths, 4 of which were of infectious etiology. Age at operation ranged from 2 months to 12 years (mean, 50 months). Weight ranged from 3.6 kg to 30 kg (mean, 12 kg). Before repair, catheterization studies revealed pulmonary hypertension in all patients with unobstructed pulmonary arteries. Pulmonary to systemic flow ratio ranged from 1.5 to 10.6 (mean, 3.5). Pulmonary vascular resistance (PVR) ranged from 0.7 to 21.7 (mean, 5.3) Wood units/m2. At repair, 14 patients had Rastelli type A anatomy, 14 had type C, and 1 patient had a variant with crossing chordae and double-outlet right ventricle (DORV). Postoperative catheterization has been done in 16 patients 2 months to 8 years (mean, 30 months) after repair. One patient had residual ventricular shunting and later underwent successful repair. A 2-year-old patient had severe mitral regurgitation and died following mitral valve replacement. One patient required a permanent pacemaker. The 21 surviving patients have been followed from 7 months to 10 years 7 months and have excellent hemodynamic status. Long-term studies are needed to assess the ultimate effect on patients with high PVR.

Flexibility and Strength Measures in Children Participating in a Cardiac Rehabilitation Exercise Program.

In brief: The purpose of this study was to evaluate the effects of a structured rehabilitation program on the strength and flexibility of children with surgically corrected congenital heart disease. Twelve children (eight boys, four girls) participated in one-hour exercise classes, two times per week for 12 weeks. Results showed minimal changes in upper extremity strength, but lower extremity strength improved 10% and 5% on the right and left sides, respectively. Flexibility improved about 25% in the lower extremities. Improvement was also noted in neck flexion, hip and oblique rotation, hamstring and low back stretch, and heel cord stretch. These findings indicate that this type of program can improve strength and flexibility in children who have undergone complex heart surgery and may help them achieve a more normal life-style.

Use of the rebreathing method in the differential diagnosis of congenital heart disease and persistent fetal circulation

The differential diagnosis of congenital heart disease from persistent fetal circulation is clinically difficult and cardiac catheterization is often needed. The development of a safe, new technique for use of the rebreathing method has allowed the determination of effective pulmonary blood flow, lung tissue volume, lung diffusion capacity and functional residual capacity in 7 critically ill, ventilator-dependent infants at the bedside. Analysis of the data revealed highly significant differences for lung tissue volume and diffusion capacity, a minimally significant difference for effective pulmonary blood flow and no difference for functional residual capacity between the groups. Use of this method allows not only attainment of clinically useful information but also permits better insight into the pathophysiology of the disease state.

Two-dimensional echocardiographic evaluation of mitral bioprosthetic function in infants and children

Two-dimensional echocardiography (2DE) was performed on 13 bioprosthetic mitral valves in 11 pediatric patients, 1 week to 46 months following implantation. Cardiac catheterization confirmed the echocardiographic findings of seven valves. The 11 normally functional bioprosthetic mitral valves by 2DE had leaflet thickness not exceeding 2.0 mm, freely moving leaflets, and normal sewing ring motion. The left atrium (LA) was enlarged only in the presence of mitral valve obstruction, atrial fibrillation, or left ventricular (LV) dysfunction. One bovine valve was stenotic due to fibrous tissue beneath normal valve leaflets; 2DE demonstrated limited leaflet motion, tissue beneath a thin valve leaflet, increase rocking motion of the sewing ring, and LA enlargement. In another patient, a bovine valve produced subaortic LV obstruction due to growth of fibrous tissue outside the stent; 2DE showed that the valve leaflets were thin and moved normally without excessive rocking of the valve ring, and identified the abnormal fibrous tissue in the LV outflow tract.

Doppler echocardiographic evaluation of tilting-disc prosthetic heart valves in children

To determine the Doppler characteristics of tilting-disc prosthetic heart valves in children, 22 children with mitral prostheses were studied 8 +/- 2 months after surgery, and 10 children with aortic prostheses were studied 37 +/- 26 months after surgery. All valves were thought to be functioning normally by clinical examination. Valve competence was interrogated and peak and mean velocities were measured by standard pulsed wave, continuous wave and color Doppler techniques. Prosthetic valve area was calculated and compared to the known valve area. Mild prosthetic valve regurgitation was present in 8 of 22 mitral and 7 of 10 aortic prostheses. For mitral prostheses, peak velocity was 192 +/- 41 cm/s, mean velocity was 118 +/- 37 cm/s and mean gradient was 7 +/- 4 mm Hg. For aortic prostheses, peak velocity was 287 +/- 88 cm/s, mean velocity was 197 +/- 59 cm/s, peak gradient was 36 +/- 21 mm Hg and mean gradient was 19 +/- 11 mm Hg. Prosthetic mitral valve area, calculated by the pressure half-time and modified Gorlin methods, correlated well with the known valve area (r = 0.89, standard error of the estimate = 0.29 and r = 0.95, standard error of the estimate = 0.21, respectively). Prosthetic aortic valve area, calculated by the modified Gorlin method, correlated well with the known valve area (r = 0.89, standard error of the estimate = 0.18). Residual valvular abnormalities are common after prosthetic valve insertion in children. Doppler estimates of prosthetic valve area correlate well with the known valve area but have a large standard error of the estimate.