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Mutations in sarcomeric proteins can lead to either hypertrophic or dilated cardiomyopathy depending on their effects on the structural and functional properties of the contractile unit of the heart. Mutations in cardiac troponin T, which binds the calcium-responsive troponin complex to α-tropomyosin, have been shown to result in cardiac hypertrophy or cardiac dilatation and heart failure, depending on the nature of the specific mutation. In this study, we report the identification of a novel cardiac troponin T mutation (A171S) leading to dilated cardiomyopathy and sudden cardiac death. In contrast to prior described mutations, the A171S mutation results in a significant gender difference in the severity of the observed phenotype with adult males (over 20 years of age) demonstrating more severe ventricular dilatation [left ventricular end diastolic dimension (LVEDD) 7.1 vs. 5.1cm; P =0.01, t test] and left ventricular dysfunction [left ventricular shortening fraction (LVSF) 21 vs. 34%; P =0.04, t test] than adult females. The described mutation substitutes a hydrophilic amino acid for a hydrophobic one in a highly conserved domain involved in the interaction between troponin T and α-tropomyosin. Interestingly, four previously described mutations within 12 amino acids of A171 lead to a hypertrophic phenotype, suggesting that further characterization of the functional consequences of the A171S mutation may lead to a better understanding of the pathophysiology of DCM and of the functional differences between HCM- and DCM-causing mutations in cardiac troponin T.

We studied three pediatric patients with protein-losing enteropathy in conjunction with univentricular hearts and right atrial to pulmonary artery anastomosis (Fontan operation) before and during heparin therapy. Each patient showed dramatic improvements in symptoms, marked elevations in serum albumin levels, and quantitative reversal of enteric protein loss within a few weeks of beginning therapy. These findings suggest that heparin may be an important treatment for this poorly understood condition.

The acute hemodynamic effects of captopril were evaluated at cardiac catheterization in 16 children (age, 0.3-18 years) with cardiomyopathy. Twelve children had congestive cardiomyopathy, whereas four had restrictive cardiomyopathy. Hemodynamic measurements were obtained 30 and 60 minutes after the oral administration of captopril (0.5 mg/kg). Blood pressures were measured in the aorta, pulmonary artery, right atrium, and pulmonary capillary wedge position; cardiac outputs were measured by the thermodilution technique. Hemodynamic data could not be obtained after the administration of captopril in one child with congestive cardiomyopathy because of an immediate, severe hypotensive response. In 11 of 12 children with congestive cardiomyopathy, cardiac index increased by 22%, from 2.3 to 2.8 l/min/m2 (p less than 0.05), and stroke volume increased by 22%, from 23 to 28 ml/m2 (p less than 0.05). Systemic vascular resistance decreased from 32 to 21 units.m2 (p less than 0.01), but the mean aortic pressure did not change significantly. In contrast, four children with restrictive cardiomyopathy had no change in cardiac output after captopril, but there was a trend toward significant arterial hypotension (mean aortic pressure decreased from 78 to 59 mm Hg). Thus, captopril acutely reduced systemic vascular resistance and increased both cardiac output and stroke volume in children with congestive cardiomyopathy. In children with restrictive cardiomyopathy, however, captopril did not affect cardiac output, but it did decrease aortic pressure. These data indicate that captopril may benefit children with a congestive cardiomyopathy but that captopril probably should not be used in children with restrictive disease.

Shone's anomaly, a congenital cardiac malformation, consists of multiple levels of left heart obstruction including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation. The prognosis for patients with Shone's anomaly is poor. To assess operative results and late outcome, we reviewed the records of 30 consecutive patients seen with Shone's anomaly at our institution between 1966 and 1989. Anatomical diagnoses in these patients were supravalvar mitral ring (22 patients), mitral valve abnormalities including parachute mitral valve, fused chordae, or single papillary muscle (26 patients), subaortic gradients (26 patients), and coarctation (29 patients). Nineteen patients had all four lesions. Other common defects were bicuspid aortic valve (19 patients) and ventricular septal defect (20). Two patients were treated medically. The other 28 patients required 84 operative procedures with 18 patients undergoing more than one procedure. Operations included coarctation repair (28 patients), mitral valve repair or replacement (11), ventricular septal defect closure (8), subaortic resection (8), and complex left ventricular outflow tract reconstruction or bypass (4). Age at first operation ranged from 7 days to 7 years (median age, 3 months). There were no operative deaths at the first operation. However, mortality rose to 24% (4/17) after the second operation. All operative deaths were secondary to severe mitral valve disease. The survivors have been followed from 1 to 16 years (mean follow-up, 6 +/- 1 years). There were no late or sudden deaths. Morbidity has included stroke (1), gastrointestinal bleeding (2), permanent heart block (1), and persistent congestive heart failure (6).(ABSTRACT TRUNCATED AT 250 WORDS)

This retrospective study was designed to evaluate the effectiveness of subcutaneous heparin therapy for the treatment of protein-losing enteropathy (PLE) associated with single-ventricle palliation and to evaluate the side effects of long-term heparin use. PLE affects 4% to 13% of Fontan operative survivors. Five-year survival after onset of PLE is only 46% to 59%. We studied a cohort of patients with single-ventricle palliation who developed PLE and were treated with subcutaneous heparin. Seventeen patients were included in the study. Symptoms of PLE appeared on average 43 months after surgical palliation. At diagnosis of PLE, mean albumin level was 2.0 +/- 0.4 g/dl. At cardiac catheterization, mean systemic venous pressure was 11.6 mm Hg. Subjective symptomatic improvement on heparin therapy occurred in 13 patients (76%). Three patients (18%) went into clinical remission. Compared with the period before initiation of heparin, there was no significant difference in the number of hospital admissions (p = 0.99) or albumin infusions (p = 0.88) during the first year of heparin therapy. Five patients had x-rays of their thoracolumbar spine, and 9 patients had bone mineral analyses; all scans were grossly abnormal. In conclusion, subcutaneous heparin therapy leads to subjective improvement of PLE symptoms in most patients; however, it does not change the need for frequent albumin infusions and does not increase the rate of remission above that for standard medical therapy.

Objective. Patient-activated transtelephonic electrocardiographic event monitors (TTMs) are often used for the evaluation of children and adolescents with suspected arrhythmias. Since their introduction 25 years ago, there has been little inquiry quantifying the usefulness of TTMs for pediatric patients. The objective of this study was to measure the utility of TTMs for children and adolescents with symptoms of a possible cardiac rhythm disturbance. Methods. Medical records of all patients who received TTMs from C.S. Mott Children’s Hospital Electrocardiography Laboratory between February 1, 1993, and October 31, 2000, were reviewed. Patients with pacemakers, known arrhythmias, or age older than 18 years were excluded. Indications for monitoring included palpitations with or without other symptoms (N = 420), chest discomfort (N = 43), and presyncope or syncope (N = 32). Results. A total of 495 studies (patient mean age: 10.2 ± 4.3 years; range: 0.1–17.9 years; 48% male) met inclusion criteria. Monitoring was performed for 1 to 1021 consecutive days (mean: 103 ± 97). Fifty-two percent (N = 257) of patients failed to transmit an electrocardiogram while experiencing symptoms. Fewer boys transmitted electrocardiograms (N = 100/238). Of 238 symptomatic patients, 15% (N = 35; mean age: 11.4 ± 4.7 years; range: 0.1–17.4 years; 51% male) had supraventricular tachycardia (SVT). No other significant arrhythmia that may warrant treatment was identified. All patients with SVT had palpitations. No patients with isolated chest discomfort, presyncope, or syncope had SVT (N = 75). SVT was documented more frequently in patients with postevent (N = 35/464) than loop recorders (N = 0/31). Of those with SVT, 71% (N = 25) and 91% (N = 33) transmitted events within 4 and 16 weeks, respectively. Follow-up for 1 to 108 months (mean: 32 ± 25; median: 26) in 53% (243 of 460) of patients without SVT uncovered a 3% (N = 7) rate of subsequent SVT detection. The overall sensitivity of the TTM test was 83% (35 of 42) for detection of SVT. The sensitivity of studies theoretically limited to 4 and 16 weeks would be 60% (25 of 42) and 79% (33 of 42), respectively. The negative predictive value of the TTM study was 99% in our patient population. The negative predictive value of tests theoretically limited to 4 and 16 weeks would be 96% and 98%, respectively. TTM studies of 2 weeks’ duration were most cost-effective in terms of total diagnostic yield. In contrast, studies of 4 weeks’ duration were most cost-efficient for SVT detection. Conclusions. TTMs are useful for the evaluation of children and adolescents with palpitations but not with isolated chest pain, syncope, or presyncope. In this study, girls were more likely to transmit events. The sensitivity of TTMs for detection of SVT was 83%. The negative predictive value of the TTM test was 99%. Monitoring for longer than 16 weeks did not increase test sensitivity. Studies of 4 weeks’ duration proved most cost-effective for SVT detection.

To determine the prevalence of malnutrition among hospitalized children with congenital heart disease by age, disease process, and clinical status.Cross-sectional, retrospective chart review.Pediatric cardiology units at a 150-bed tertiary care teaching hospital in Ann Arbor, Mich.Patients (n = 160) were randomly selected from consecutive admissions to the Pediatric Cardiology and Thoracic Surgery Services during a 1-year period.None.Acute and chronic malnutrition, assessed by comparing the patients' weight and height with established means.Acute and chronic malnutrition occurred in 33% and 64% of the patients, respectively. Age, diagnostic category, and symptoms were associated with malnutrition. Eighty percent of infants presented with acute malnutrition compared with 18% of patients of other ages (P.001). Malnutrition affected 60% of patients with left-to-right shunts, 53% of patients with complex heart disease, and no patients with primary rhythm disturbances. Acute malnutrition affected 11% and chronic malnutrition affected 50% of patients with left-sided heart obstruction. Acute or chronic malnutrition occurred in 70% or more of patients with cyanosis and/or congestive heart failure but in only 30% of patients with neither (P.001).Malnutrition in hospitalized children with congenital heart disease remains common, highlighting the importance of nutritional screening and intervention.

Today's health care environment prompted implementation of a case management model by pediatric nurse practitioner clinical nurse specialists to promote the organization of resources for optimal care of children with heart disease. Evaluation of this pilot program suggests that achievement of expected outcomes within an appropriate length of stay was facilitated, that parents were ready for discharge, and that readmissions were infrequent. In addition, patient and system variances resulting in delay of discharge and discharge preparation needs were identified. The pediatric nurse practitioner as case manager may have a significant impact on the quality and cost of care for hospitalized children.

BACKGROUND Percutaneous balloon valvuloplasty has proven to be acutely effective in the treatment of congenital valvar aortic stenosis; however, the intermediate- and long-term effectiveness of the procedure remain to be documented. METHODS AND RESULTS To assess the intermediate-term effectiveness of balloon valvuloplasty, repeat catheterization was performed in 27 of 30 children 1.7 +/- 0.1 years after balloon valvuloplasty for congenital aortic stenosis (AS). In 33 children the peak AS gradient was reduced acutely by 55% from 77 +/- 4 to 35 +/- 3 mm Hg (p less than 0.001), and left ventricular systolic pressure was reduced from 176 +/- 4 to 138 +/- 4 mm Hg (p less than 0.001). Despite a technically adequate valvuloplasty procedure, three patients had inadequate relief of obstruction and required complex surgical intervention. Twenty-seven of the 30 patients available for late reevaluation (90%) enrolled in the follow-up study. The peak AS gradient remained significantly reduced compared with that present before valvuloplasty (29 +/- 3 versus 77 +/- 4 mm Hg, p less than 0.001). Furthermore, there was no difference in peak AS gradient at follow-up compared with that immediately after valvuloplasty. The greatest increase in gradient at reevaluation was 14 mm Hg. Twenty of 27 patients (74%) had no change in the degree of aortic insufficiency at follow-up compared with that present before valvuloplasty. At follow-up, 16 patients had no aortic insufficiency at all, and only two had moderate-to-severe (3-4+) insufficiency. Femoral artery injury was documented in four patients, three of whom were under 12 months of age at valvuloplasty. CONCLUSIONS Balloon aortic valvuloplasty provides safe and effective intermediate-term gradient relief without early restenosis in children and adolescents with congenital AS.

We defined the natural history of scoliosis in congenital heart disease (CHD) with respect to the risk of progression and indications for treatment. We reviewed radiographs of 48 children with CHD and scoliosis. Thirty-seven had developmental scoliosis (77%), and 11 (23%) had congenital scoliosis. Children with congenital scoliosis can be managed in the same way as children without CHD. Developmental curves less than 30 degrees in children aged greater than 10 years showed no progression. Developmental curves greater than 30 degrees with onset in children aged less than 10 years and progression greater than 9 degrees per year require treatment.

In an attempt to determine if percutaneous arterial catheterization rather than open arteriotomy or the use of an antiplatelet agent, aspirin, would reduce the incidence of arterial thrombosis after cardiac catheterization, 95 children were studied. After measuring the amplitude of pulsations by oscillometry on the day of admission, the children were randomly divided into two groups. One received aspirin 15 mg. per kilogram of body weight per dose for 5 doses and the other served as a control. Method of arteriotomy—percutaneous or open surgical incision—was left to the discretion of the catheterizer. Repeat oscillometric measurements were obtained before discharge. Percutaneous catheterization was associated with a significantly fewer number of diminished pulses (p =

The most recent postoperative echocardiographic examinations of all children who underwent arterial switch repair of transposition of the great arteries from August 1985 to December 1987 were reviewed. The patients included 35 children whose age at operation was 12 ± 16 days and whose weight was 3.6 ± 0.4 kg. Thirty-three patients are alive and well; 1 died intraoperatively and 1 died immediately postoperatively. The time of the follow-up echocardiographic examination ranged from 1 day to 2.5 years (mean 9.2 months) with 11 patients examined >1 year after surgery. Complete examination of the repair site was possible in all patients. Echocardiographic visualization of distortion of the great arteries at the suture lines was seen in all patients; however, Doppler evidence of hemodynamically significant obstruction at the repair site was uncommon. On Doppler examination in the surviving 33 patients, 16 had no supravalvular pulmonary stenosis and 14 had mild to moderate supravalvular pulmonary stenosis with peak systolic pressure gradients ranging from 16 to 56 mm Hg (mean 31). Three patients had severe supravalvular pulmonary stenosis and peak systolic pressure gradients of 66, 74 and 77 mm Hg (2 have had reoperation, 1 is awaiting surgery). On Doppler examination, 4 patients had mild supravalvular aortic stenosis with peak systolic gradients ranging from 10 to 29 mm Hg. Doppler gradients were confirmed in 10 patients who had catheterization 12 ± 3 months after surgery. Three patients had mild pulmonary regurgitation by Doppler examination, 5 had mild aortic regurgitation, 4 had mild tricuspid regurgitation and 2 had mild mitral regurgitation. In all 33 patients, ventricular dimensions, left ventricular shortening fraction (42 ± 6%) and rate-corrected mean velocity of circumferential fiber shortening (1.25 ± 0.24 circumference/s) were normal.

Twenty-nine children were evaluated prospectively for the presence of an aortic aneurysm at the repair site 1 to 19 years after patch aortoplasty repair of coarctation of the aorta. In each child, noninvasive evaluation included a chest X-ray film, computed tomography of the chest and two-dimensional echocardiography. The presence and size of an aortic aneurysm were determined quantitatively by measuring the ratio of the diameter of the thoracic aorta at the repair site to the diameter of the aorta at the diaphragm (aortic ratio). An aortic ratio of ≥1.5 was judged abnormal and was shown to be significantly greater than the aortic ratio of a normal control group. An aortogram was obtained in each child if any noninvasive screening test was found to be abnormal. As assessed by the aortogram, the prevalence of aortic aneurysm was 24% in this patient group. The sensitivity of echocardiography and chest computed tomography for detecting an aneurysm was 71% and 66%, and the specificity 76% and 85%, respectively. The chest X-ray film was 100% sensitive and 68% specific in determining the presence of an aneurysm. Although the data are not statistically significant, they suggest that children undergoing patch aortoplasty as the primary procedure (rather than a reoperation after earlier resection), and children in whom a Dacron patch is utilized may be at increased risk for aneurysm formation. Thus, in a prospective manner, this study has documented that aortic aneurysm occurs commonly after patch aortoplasty for coarctation in childhood, that the chest X-ray film provides a sensitive screening test and that the aneurysm may be evaluated quantitatively (by measuring the aortic ratio) with echocardiography or chest computed tomography.

The development of systemic hypertension, in an eight-year-old girl, after resection of a large arteriovenous malformation, is described. The hypertension was related to an elevated plasma renin activity and was controlled with propranolol. A possible mechanism for the rise in plasma renin activity is postulated.

To determine the diagnostic value of exerciseinduced R-wave changes in adolescents with congenital heart disease, the responses of 50 adolescents without significant heart disease were compared with those of 72 patients with either a left ventricular (LV) pressure or volume overload lesion. Among the pressure overload group, 24 patients had valvular aortic stenosis (AS) and 27 had coarctation of the aorta. The volume overload group included 12 patients with mitral regurgitation (MR) and 9 with aortic regurgitation (AR). Severity of the cardiac lesion was assessed using cardiac catheterization in patients with AS, physical examination in patients with coarctation of the aorta and clinical or angiographiec criteria, or both, in patients with valvular regurgitation. The R wave was measured in 10 consecutive QRS complexes in leads II, aVF and V 5 at rest, maximal exercise and 1-minute recovery. At maximal exercise, control subjects had a mean decrease in amplitude (ΔR) of −3.6 mm (p

A retrospective review of 149 episodes of bacterial endocarditis (BE) in 141 patients under 25 years of age, at The Children's Hospital Medical Center from 1933 through June of 1972, demonstrates increasing survival and a distinct change in the frequency of underlying congenital heart disease and rheumatic heart disease (RHD). Certain forms of congenital heart disease such as tetralogy of Fallot, small ventricular septal defect, and aortic stenosis are at particular risk for BE. Following BE, patients with ventricular septal defect and tetralogy of Fallot have less morbidity and higher survival rates than children with aortic outflow lesions. over the entire time period, alpha Streptococcus is the most common pathogen and Staphylococcus aureus, second most frequent organism. Surgical correction in patients with congenital heart disease may offer the best form of prevention.

The etiology of chronic congestive heart failure (CHF) after repair of tetralogy of Fallot was determined in 102 patients consecutively catheterized 1 to 12 years postoperatively. Chronic CHF was observed in 36/102 patients. The most prevalent abnormality leading to congestive failure (31/36) was a large residual ventricular septal defect alone or in combination with other lesions. All postoperative patients with pulmonary to systemic flow ratios greater than 2:1 (25/102) had congestive failure and evidence of biventricular dysfunction. Significant tricuspid regurgitation (N = 11) and persistent systemic to pulmonary artery shunts (N = 6) contributed to volume overload and congestive failure in the patients with large residual ventricular septal defect. Isolated severe residual right ventricular outflow tract obstruction was a common cause of chronic CHF. Pulmonary artery hypertension was present in 20/36 patients with CHF. The increased pulmonary pressure was not wholly due to an increased pulmonary flow since 7/20 patients had pulmonary vascular resistance greater than 3 mm Hg/L/min/m2. Our findings indicate that persistent or chronic congestive heart failure in postoperative tetralogy of Fallot patients requires bilateral cardiac catherterization since an identifiable and surgically correctable lesion is nearly always present.

No data exist concerning the total sinoatrial conduction time (TSACT) in children that compare values determined by the atrial extrastimulation technique (TSACTS) with those generated by the atrial pacing method (TSACTN). In this study, TSACT in 55 patients, age 0.2-18.5, was measured using both techniques. TSACTN was performed at a mean 90% (TSACTN-90) (n = 32) or a mean 95% (TSACTN-95 and (n = 38) of sinus cycle length (SCL). When data generated during determination of TSACTN-90 and TSACTS were compared, SCL and recovery cycle length (REC) were similar for both techniques. Likewise, TSACTS (128 +/- 40 ms) and TSACTN-90 (126 +/- 74 ms) were not significantly different. Coefficient of correlation was r = 0.82, p less than 0.001. Chi-square analysis demonstrated a strong association of normal and abnormal values between TSACTS and TSACTN-90. In contrast, when values generated during TSACTN-95 and TSACTS were compared, TSACTS exceeded TSACTN-95 (137 +/- 38 vs 105 +/- 58 ms; p less than 0.001). Values for SCL and REC were similar while correlation between TSACT determined by the two techniques remained strong (r = 0.82, p less than 0.001). Despite a good correlation between TSACTN-90 and TSACTS, individual differences in magnitude and direction were noted between the two techniques. In summary, TSACTN-90 approximates TSACTS in children. TSACTN-90 is preferable to TSACTN-95, probably due to more complete sinus node capture during atrial pacing. However, the behavior of the sinus node in response to extrastimuli (single or train) precludes favoring one technique over the other. More precise evaluation of sinoatrial conduction will require direct recording of sinus node activity.

To assess left ventricular (LV) diastolic filling in children with pressure overload hypertrophy, 12 patients with LV outflow obstruction (7 with aortic valve stenosis and 5 with aortic coarctation) and 12 healthy, age-matched control subjects were examined. Each child underwent M-mode echocardiography and pulsed Doppler examination of the LV inflow. The patients with LV outflow obstruction had cardiac catheterization and balloon angioplasty. Their echo/Doppler examinations were performed in the catheterization laboratory before and immediately after balloon angioplasty. From the M-mode echocardiogram, the LV cavity dimensions and wall thicknesses, LV mass and shortening fraction were measured. The following measurements were made from the Doppler recording: peak velocities at rapid ventricular filling (peak E) and during atrial contraction (peak A), ratio of peak E to peak A velocities, total area under the Doppler curve, percent of the total Doppler area occurring in the first one-third of diastole (0.33 area fraction), percent of the total area occurring under the E wave (E area fraction), percent of the total area occurring under the A wave (A area fraction) and the ratio of E area to A area.(ABSTRACT TRUNCATED AT 250 WORDS)

Left ventricular (LV) apical aneurysms were observed in 16 of 50 (32%) children (average age 8 years) consecutively catheterized after surgical repair of congenital heart disease. The LV apex was vented by a sump during cardiopulmonary bypass in each. The aneurysms varied in size, but were generally small. Average dimensions were 7.5 X 6.8 mm in the anteroposterior projection and 8.9 X 5.7 mm in the left anterior oblique projection. The LV apex wall was thinner in patients with aneurysms than in age- and lesion-matched controls. All of the LV aneurysm patients were asymptomatic during average follow-up of 4 years. Nevertheless, such aneurysms are anticipated to represent a potential source of cardiovascular complications and, when possible, alternate methods for venting the left ventricle are recommended.

Balloon angioplasty was attempted in 13 children with peripheral pulmonary arterial (PA) stenosis. In 5 patients, angioplasty was successful in relieving the peripheral PA stenosis as judged by an increase in PA size of more than 75% over the predilatation size and a more than 50% reduction in the distal PA to main PA peak systolic pressure gradient. Each child has been followed for 6 to 30 months. All remain well, without signs of subsequent deterioration, and follow-up angiograms in 2 patients (at 10 and 12 months) showed persistence of anatomic and hemodynamic improvement. In 8 patients, angioplasty was unsuccessful: In 4 patients, stenosis at the site of a previous systemic-to-PA shunt could not be dilated and in 4 patients, angioplasty could not be performed because of technical difficulties. Thus, we could not dilate the stenosis in more than 60% of the patients; we also had a significant complication with the angioplasty procedure (perforation of a distal branch of the right pulmonary artery). Thus, although balloon angioplasty was not effective in all patients, it did provide significant improvement in some patients in whom traditional operative management is usually unsuccessful.

The acute hemodynamic effects of verapamil were evaluated in nine children with hypertrophic cardiomyopathy. Verapamil, 0.1 mg/kg, was administered as an i.v. bolus over 2 minutes, followed by a 20-minute continuous infusion of 0.007 mg/kg/min. Hemodynamic measurements were obtained at rest in nine patients and at maximal supine bicycle exercise in seven before and 15 minutes after verapamil. At rest, verapamil increased the mean cardiac output from 3.3 +/- 0.9 to 3.7 +/- 0.9 l/min/m2 (+/- SD) (p less than 0.02) and decreased left ventricular end-diastolic pressure from 19.3 +/- 8.1 to 14.5 +/- 6.9 mm Hg (p less than 0.006). In six patients with resting left ventricular outflow tract obstruction, the systolic pressure gradient decreased from 17.5 +/- 7.2 to 5.2 +/- 4.5 mm Hg (p less than 0.04). Repeat supine bicycle exercise testing after verapamil showed increases in total work performed (1743 +/- 1284 to 3168 +/- 1643 kg-m, p less than 0.006) and maximal cardiac index during exercise (6.5 +/- 1.3 to 7.8 +/- 1.8 l/min/m2, p less than 0.05), and decreases in maximal exercise left ventricular end-diastolic pressure (29.1 +/- 10.1 to 19.3 +/- 10.4 mm Hg, p less than 0.002) and left ventricular systolic outflow tract gradient (31.2 +/- 10.5 to 1.75 +/- 1.7 mm Hg, p less than 0.04). These results suggest that verapamil may be an effective therapeutic agent for the treatment of hypertrophic cardiomyopathy in children.

The course and prognosis of 16 infants and children with sick sinus syndrome associated with cardiac surgery is reviewed. The dysrhythmia was observed most ofter after extensive atrial reconstructive surgery in patients with transposition of the great arteries and with atrial septal defect. In 12 (75%) of the patients, sick sinus syndrome was detected in the immediate postoperative period. Tachyarrhythmias and bradyarrhythmias were presnet in 12 and isolated bradyarrhythmias in four. Temporary pacing was used in two and permanent pacing was required in five. Death in two non-paced patients was attributable to arrhythmias. Postmortem examination in one patient revealed a suture in the sinoatrial node. Careful attention to the anatomy of the sinoatrial node, its artery and the internodal tracts during surgery may prevent the development of sick sinus syndrome. In patients with dysrhythmia, a careful search should be undertaken to document the abnormal rhythm with the use of Holter monitoring. The insertion of a pacemaker is indicated in patients with tachyarrhythmias requiring cardioversion or antiarrhythmic drug therapy and those without adequate lower escape mechanisms.

To assess the usefulness of the Doppler mean gradient as a noninvasive indicator of the need for intervention, 33 children (ages 3 months to 20 years) with valvular aortic stenosis (AS) underwent a 2-dimensional and Doppler echocardiographic examination a median of 1 day before cardiac catheterization. The clinical decision for intervention was based on finding a catheterization peak-to-peak pressure gradient of greater than 75 mm Hg or from 50 to 75 mm Hg in the presence of symptoms or an abnormal exercise treadmill test result. Of the 33 patients, 23 required intervention. The decision for intervention was compared to the Doppler mean gradient, and the Doppler peak and mean gradients were compared to the catheterization peak-to-peak gradient. All 12 patients with a Doppler mean gradient greater than 27 mm Hg had intervention and had a catheterization peak-to-peak gradient of greater than or equal to 75 mm Hg. All 3 patients with a Doppler mean gradient less than 17 mm Hg had no intervention and had a peak-to-peak gradient less than 50 mm Hg. The remaining 18 patients with Doppler mean gradients between 17 and 27 mm Hg comprised an intermediate group in whom the Doppler mean gradient alone did not predict the need for intervention. From a chi-square table, a Doppler mean gradient greater than 27 mm Hg predicted the need for intervention with 100% specificity (no false positives) and 52% sensitivity (11 false negatives).(ABSTRACT TRUNCATED AT 250 WORDS)

We prospectively examined the usefulness of electrocardiography, chest radiography, and M-mode echocardiography in discriminating between the presence and absence of heart disease in 280 children older than one month and newly referred for evaluation of a heart murmur. After taking a history and performing a physical examination but before reviewing diagnostic tests, we categorized the children as having "no heart disease" (142), "possible heart disease" (34), or "definite heart disease" (104). Among the children initially thought to have no heart disease, the diagnosis was changed after a review of diagnostic tests in eight--three with mitral-valve prolapse, two with possible cardiomyopathy, and three with no heart disease on follow-up. Among those initially thought to have possible heart disease, the tests changed the diagnosis to definite heart disease in four, of whom only one had heart disease (mitral-valve prolapse) on follow-up. In no case did a review of tests change the diagnosis of definite heart disease. We conclude that the results of diagnostic tests are unlikely to change the clinical diagnosis of no heart disease or definite heart disease, when made by a qualified pediatric cardiologist in children newly referred for evaluation of a heart murmur.

Surgical repair was performed on 8 patients with tetralogy of Fallot and congenital or acquired absence of the left pulmonary artery. Prior palliative systemic-pulmonary artery shunts had been performed in the 5 patients with acquired absence of the pulmonary artery, and in none was repair of the damaged pulmonary artery possible. A valve-containing conduit (Hancock) was used in each patient to prevent pulmonary valvular regurgitation during the early postoperative period. All patients survived operation and have exhibited marked symptomatic and hemodynamic improvement. This contrasts with the poor results of corrective operation previously reported in patients in this diagnostic category. We believe that the present improved results are due in large part to the valved conduit, and we therefore recommend its use during repair in children or adults with tetralogy and a single pulmonary artery.

To assess the flow characteristics of homograft valved conduits in the immediate postoperative period, 69 children with 71 homograft conduits underwent 2-dimensional and Doppler echocardiographic examination at 1 to 40 days (mean 8) after surgery. Of the 71 conduits studied, 19 were aortic and 52 were pulmonary homograft valved conduits. Two aortic homograft valved conduits were inserted in the aortic position, whereas all remaining homografts were placed in the pulmonary position. On the immediate postoperative echocardiogram, 25 (35%) of the conduit valves had no regurgitation and 44 (62%) had 1+ (mild) regurgitation. Two pulmonary valved conduits (3%) in the pulmonary position had 2+ (moderate) regurgitation and right ventricular dimensions >95% for body surface area. The peak velocity across the homograft valve was normal ( 2.6 m/s in the immediate postoperative period. To assess the fate of homograft valved conduits in the intermediate-term follow-up period, 38 children with 38 conduits had a repeat echocardiogram at 6 to 25 months (mean 15 ± 6) after surgery. Of the 38 conduits examined, 10 (26%) had no regurgitation, 25 (66%) had 1+ regurgitation and 3 (8%) had 2+ regurgitation. Progression of the amount of regurgitation occurred in 11 (29%) patients. At the follow-up examination, peak velocity was ≤1.4 m/s across 34 conduit valves, between 1.4 and 2.6 m/s across 3 valves and >2.6 m/s across 1 valve. This latter patient had a 3.2-m/s jet across the proximal insertion of the conduit and no further increase across the valve itself. Thus, no patient had evidence of conduit valve stenosis. Of the 38 patients, 9 had Doppler evidence of obstruction at the conduit insertion (8 at the distal end and 1 with the aforementioned proximal obstruction). Most obstructions were of mild to moderate severity and only 2 patients (5%) had severe conduit stenosis. Thus, in the immediate postoperative period, normally functioning homograft valved conduits frequently have mild regurgitation (62%) and rarely have moderate or severe regurgitation (3%), with peak velocities reaching Thus, in the immediate postoperative period, normally functioning homograft valved conduits frequently have mild regurgitation (62%) and rarely have moderate or severe regurgitation (3%), with peak velocities reaching

Transluminal balloon valvuloplasty was used in the treatment of congenital valvar pulmonary stenosis in 19 children, aged 5 months to 18 years. The right ventricular (RV) systolic pressure and RV outflow tract gradient decreased significantly immediately after the procedure (95 +/- 29 vs 59 +/- 14 mm Hg, p less than 0.01, and 78 +/- 27 vs 38 +/- 13 mm Hg, p less than 0.01). Seven of these patients were evaluated at cardiac catheterization 1 year after balloon valvuloplasty. No significant change occurred in RV systolic pressure or RV outflow tract gradient at follow-up evaluation compared with measurements immediately after balloon valvuloplasty (60 +/- 5 mm Hg vs 56 +/- 12 mm Hg and 39 +/- 5 vs 38 +/- 10 mm Hg). In addition, follow-up evaluation was performed using noninvasive methods and included electrocardiography (n = 13), vectorcardiography (n = 11) and Doppler echocardiography (n = 11) Doppler echocardiography in 11 patients 15 +/- 9 months after balloon valvuloplasty showed a continued beneficial effect with a mild further decrease in RV outflow tract gradient. Thus, balloon valvuloplasty is effective in the relief of pulmonary stenosis.

The incidence and possible causes of paradoxical hypertension were evaluated in eight children who underwent balloon dilatation and seven children who underwent surgical repair of coarctation of the aorta. Both procedures resulted in a significant reduction in the coarctation gradient. Both systolic and diastolic blood pressures increased in the surgical group after repair, whereas systolic pressures decreased and diastolic pressures remained unchanged after balloon angioplasty. In the surgical group, but not in the balloon angioplasty group, plasma catecholamines and plasma renin activity rose during the first 2 days after relief of the coarctation. The data presented in this report support the hypothesis that the sympathetic nervous system and the renin angiotensin system are important mediators of the paradoxical hypertension that occurs after surgical repair of coarctation. Furthermore, balloon angioplasty of coarctation of the aorta does not stimulate either system and thus paradoxical hypertension is not a complication of this procedure.

To assess the long-term results of left ventricular outflow tract reconstruction utilizing an apical left ventricular to aortic valved (porcine) conduit the clinical and hemodynamic data were reviewed from 24 patients who had placement of an apico-aortic conduit. Eighteen of the patients are asymptomatic and taking no cardiac medications. Three patients were reoperated on, one patient 1.5 years after his original operation for subacute bacterial endocarditis and two patients 3 to 4 years after their original operation for severe conduit valve insufficiency. None of the patients is taking anticoagulants and no thromboembolic events have occurred. Postoperative catheterization has been performed 1 to 1.5 years (mean 1.2) after repair in 15 of 21 patients. The rest left ventricular outflow tract gradient has decreased from 102.5 +/- 20 mm Hg preoperatively to 14.8 +/- 9.9 mm Hg postoperatively (probability [p] less than 0.001). Some degree of conduit obstruction was demonstrated by catheter passage in 11 of the 15 patients. In these 11 patients, the obstruction occurred at three distant sites: at the egress of the left ventricle in 9, at the porcine valve in 5 and at the aortic to conduit junction in 1. Isometric exercise in five and supine bicycle exercise in six patients increased the left ventricular outflow tract gradient by 2.5 +/- 1.1 and 20.8 +/- 11.8 mm Hg, respectively, despite an increase in cardiac index of 1 +/- 0.3 and 3.7 +/- 0.4 liters/min per m2, respectively. The data suggest that a left ventricular to aortic conduit is an effective form of therapy for severe left ventricular outflow tract obstruction.

Brain abscess remains a serious complication of congenital heart disease. To determine risk factors for brain abscess in patients with this condition, data in 26 cases of cyanotic congenital heart disease and brain abscess studied between 1960 and 1973 were reviewed and compared with data in a control group with cyanotic congenital heart disease without brain abscess. Brain abscess occurred in 2 percent of the population with cyanotic congenital heart disease. Tetralogy of Fallot and dextrotransposition of the great arteries accounted for 81 percent (21 of 26) of the cases. The mean arterial oxygen saturation in the patients with brain abscess was 75 percent compared with 86 percent in the control patients (P < 0.01). Among the patients with brain abscess, the survivors (16 of 26) had a mean oxygen saturation of 81 percent whereas those who died had a mean oxygen saturation of 64 percent (P = 0.01). The overall mortality rate in the patients with brain abscess was 38 percent (10 of 26). Among survivors, 54 percent (8 of 15) had complete recovery and 46 percent (7 of 15) had residual neurologic impairment. We conclude that the morbidity and mortality of brain abscess are inversely related to oxygen saturation levels. Since brain abscess is exceedingly rare in patients under age 2 years, corrective surgery before this age would probably reduce the incidence of brain abscess in patients with congenital heart disease.

Summary: Quantitative morphometric studies were performed on the pulmonary arteries of two newborns who died at 1 and 3 days of age with both transposition of the great arteries and persistence of the fetal circulation. Similar studies were performed on two normal control subjects (age 2 and 6 days), one newborn infant (age 1 day) with secondary persistence of the fetal circulation, and one newborn infant (age 2 days) with isolated D-transposition of the great arteries. Morphometric data, summarized herein, showed greatest mean percent wall thickness in the patients with persistent fetal circulation either alone or with D-transposition of the great arteries, as compared to those without it. When wall thickness was related to external diameter of the vessel, the greatest difference in thickness was observed in the smallest vessels (

To compare the ability of high pulse repetition frequency and continuous wave Doppler echocardiography to detect the peak velocity of a jet flow disturbance and to predict pressure gradients accurately, two groups of children with valvular or congenital heart disease were examined using both Doppler techniques. The use study group included 84 children or adolescents (aged 1 day to 19 years) who underwent examination in the echocardiography laboratory with both Doppler techniques in a randomized sequence. The peak velocity recorded with high pulse repetition frequency Doppler echocardiography was compared with the peak velocity recorded with the continuous wave technique. The accuracy study group included 41 children or adolescents (aged 1 day to 16 years) who underwent examination with both Doppler techniques at the time of cardiac catheterization. Doppler pressure gradients were calculated from the peak velocity using the simplified Bernoulli equation and were compared with peak instantaneous gradients and peak to peak gradients measured at catheterization. In the use study, a high correlation was found between peak velocities detected by high pulse repetition frequency and continuous wave Doppler echocardiography (r = 0.94, SEE = 0.28 m/s). In the accuracy study, close correlations were found between measured peak to peak pressure gradients and pressure gradients calculated from continuous wave (r = 0.95, SEE = 7.9 mm Hg) and high pulse repetition frequency Doppler echocardiography (r = 0.94, SEE = 8.7 mm Hg). Also, close correlations were found between measured peak instantaneous gradients and pressure gradients calculated from continuous wave (r = 0.96, SEE = 7.1 mm Hg) and high pulse repetition frequency Doppier echocardiography (r = 0.95, SEE = 7.5 mm Hg). Thus, in children and adolescents, no difference was found in the ability of the two Doppler techniques to detect the peak velocity of a jet flow disturbance. Both Doppler techniques were equally accurate in their ability to predict pressure gradients measured at cardiac catheterization.

The pathogenesis of paradoxical hypertension after resection of coarctation of the aorta was investigated by comparing the course of seven children undergoing repair of coarctation with five acyanotic children undergoing elective cardiovascular surgery. During the first 24 hours after surgery, all coarctation patients demonstrated a rise in systolic blood pressure (35 +/- 15.5 mm Hg; P less than 0.001), a significant depression in cold pressor test response, and only a slight elevation in plasma renin activity. In the next 24-72 hours, coarctation patients developed a rise in diastolic blood pressure (26.8 +/- 10.6 mm Hg; P less than 0.001), plasma renin activity (22.9 +/- 10.2/ml/hr; P less than 0.001) and fluid retention. By contrast, control patients had no significant postoperative changes. Abdominal pain occurred in five coarctation patients during the period of maximal plasma renin activity. The data suggest that the sympathetic nervous system may be responsible for the initial phase of hypertension after coarctation resection and that the renin angiotension system plays a major role in the second phase of hypertension and in the pathogenesis of mesenteric arteritis.

Percutaneous balloon pulmonary valvuloplasty was performed in seven children with moderate to severe valvular pulmonary stenosis (right ventricular to pulmonary artery pressure gradient greater than 50 mm Hg). All patients experienced a decrease in right ventricular peak systolic pressure from 108 +/- 30 to 60 +/- 5.6 mm Hg (p less than 0.001), decrease in right ventricular to pulmonary artery gradient from 90.1 +/- 30 to 38.8 +/- 5 mm Hg (p less than 0.001) and increase in pulmonary valve area from 0.33 +/- 0.06 to 0.55 +/- 0.15 cm2/m2 (p less than 0.001). In the two patients who underwent supine bicycle exercise before and after valvuloplasty, a significant decrease in both the maximal right ventricular peak systolic pressure (212 to 140 and 175 to 125 mm Hg, respectively) and in right ventricular to pulmonary artery peak pressure gradient (185 to 110 and 151 to 85 mm Hg, respectively) occurred. All patients tolerated the procedure well and no serious complications were observed. It is concluded that percutaneous balloon valvuloplasty is a safe and effective method for relief of right ventricular obstruction due to moderate or severe valvular pulmonary stenosis. However, long-term results remain unknown.

To examine the excitation of the double chamber right ventricle and the possibility that it results from a displaced, hypertrophied moderator band, seven patients with double chamber right ventricle were studied. All seven had pre- and postoperative electrocardiograms. Intraoperative right ventricular epicardial maps were obtained in three; right ventricular endocardial activation maps performed at postoperative catheterization were obtained in four. In the three patients studied at operation the breakthrough point of right ventricular epicardial activation was demonstrated in a normal inferior location well below the obstructing muscle band. Two patients with right bundle branch block after operation and two others with only right ventricular conduction delay on postoperative electrocardiogram demonstrated high normal right ventricular time with prolongation of activation in the right ventricular outflow or inflow region, or both, suggesting only peripheral injury. These data suggest that activation of the double chamber right ventricle is similar to that of the normal heart. In addition, the observed normal activation sequence militates against the hypothesis that the moderator band is the obstructing bundle.

Between 1955 and 1972, eight children receiving mediastinal irradiation for intrathoracic malignancy developed constrictive pericarditis. Six of these followed irradiation for Hodgkin's disease and represent 7% of 86 children receiving irradiation for that disease. In patients with Hodgkin's disease, the development of constrictive pericarditis was related to the dose of radiation and the use of orthovoltage technique. Constrictive pericarditis has not yet developed among the 44 children radiated by supravoltage technique. Symptoms of constrictive pericarditis occurred an average of 15 months (4/12 to 3 2/12 years) after irradiation and were preceded in five of the eight patients by acute pericarditis. Prominent clinical findings included dyspnea, Kussmaul sign, pulsus paradoxicus, hepatomegaly, cardiomegaly and electrocardiographic evidence of low QRS voltages with ST-T wave changes. Pericardiectomy was performed in six patients and resulted in immediate but only transient symptomatic relief. There was no operative mortality. Death occurred in six of the eight patients (1 5/12 to 6 6/12 years following irradiation) due to cardiac disease in three, progression of the malignancy in two and infection in one. In addition to constrictive pericarditis, extensive myocardial fibrosis and involvement of the antero-lateral papillary muscle and chordae tendineae of the mitral valve were demonstrated at postmortem examination. These factors may explain the progression of cardiac disease despite an initial symptomatic improvement following pericardiectomy.

Thirty-four (8%) of 453 infants and children with urinary system malforma tions had cardiovascular malformations. Cardiac defects were seen frequently in Potter syndrome, unilateral renal agenesis, and horseshoe kidney. The most frequently encountered types of heart defects were ventricular septal defect (33%), endocardial cushion defect (15%), tetralogy of Fallot (12%), and patent ductus arteriosus (12%).

Patients undergoing repair of coarctation of the aorta often have self-limited but severe hypertension in the first week after surgery (paradoxical hypertension). We conducted a controlled trial of treatment with propranolol before repair of coarctation of the aorta in 14 children to determine whether the drug would prevent paradoxical hypertension. Seven patients were randomly assigned to receive propranolol for two weeks before surgery and throughout the first postoperative week, and seven patients were assigned to receive standard postoperative care. Both groups had a similar significant (P less than 0.05) increase in the plasma norepinephrine level in response to surgery; however, when compared with no treatment, treatment with propranolol reduced not only the rise in systolic (P = 0.004) and diastolic (P = 0.003) blood pressure but also the postoperative increase in plasma renin activity (P less than 0.01). We conclude that prophylactic propranolol can prevent paradoxical hypertension and should therefore become a routine part of the operative care of patients with coarctation of the aorta.

Summary The course and prognosis of 208 patients with an ascending aorta to pulmonary artery anastomosis is reviewed. Mortality rate during, or within one month, of surgery was 24 per cent (50/208) and late mortality rate, prior to repair, was 10 per cent (21/208). An additional 5 per cent (10/208) died during subsequent intracardiac repair. Congestive heart failure developed in 25 per cent (53/208), pulmonary artery hypertension in 17 per cent (12/72), and pulmonary vascular obstruction in 6 per cent (4/72). An increase in orifice size of the stoma with time was documented in eight patients. Additional subsequent palliative surgery was required in 22 per cent (45/208). Mortality rate was directly related to age to operation and was highest in neonates less than one week of age. In infants with tetralogy of Fallot, a preliminary comparison of mortality rate between palliative surgery and primary repair clearly suggests that the latter is the preferred method of treatment.

The Valsalva maneuver was performed during cardiac catheterization in 9 patients who had undergone a Fontan operation (Fontan group) and in 10 control subjects. The Fontan group had higher right atrial (RA) pressure (16 +/- 1 vs 4 +/- 0.5 mm Hg, p less than 0.001), lower cardiac index (2.5 +/- 0.1 vs 3.7 +/- 0.2 liters/min/m2, p less than 0.05), lower stroke index (32 +/- 2 vs 44 +/- 2 ml/beat/m2, p less than 0.05), and higher systemic vascular resistance (31 +/- 1 vs 24 +/- 1 units, p less than 0.05) than the control group. Four patients in the Fontan group had a normal 4-phase Valsalva response, and 5 did not (p less than 0.05). RA pressure was similar in those who responded normally and those who did not (16 +/- 0.5 vs 17 +/- 1 mm Hg), whereas in those who responded normally cardiac index was higher (2.9 +/- 0.2 vs 2.2 +/- 0.1 liters/min/m2, p less than 0.05), stroke index was higher (36 +/- 1 vs 28 +/- 1 ml/beat/m2, p less than 0.05), and systemic vascular resistance was lower (28 +/- 1 vs 31 +/- 1, p less than 0.05). It is concluded that cardiac output is a better predictor of a normal response to the Valsalva maneuver than RA pressure in patients after the Fontan procedure. A normal response to the supine Valsalva maneuver suggests a cardiac index greater than 2.4 liters/min/m2 and stroke index greater than 31 ml/beat/m2.

Clinical and pathologic findings in a 13 13 12 year old girl with coronary artery disease of an apparently unique type are described. Necropsy revealed a generalized arterial disease characterized by degeneration and calcification of the media with focal thickening of the intima. Other findings included antinuclear antibodies, low serum copper and ceruloplasmin levels, anetoderma and pulmonary granulomas. These findings suggest the possibility of an underlying autoimmune or metabolic disorder.

The development of hemodynamically significant muscular subaortic stenosis in association with pulmonary artery banding is described in one patient with D-transposition of the great arteries, and another with interrupted aortic arch. It is postulated from these and previously reported cases that hypertrophy of the conal septum (crista supraventricularis), whether subpulmonary or subaortic, congenitally displaced or grossly normal, may follow pulmonary artery banding and result in progressive subaortic outflow obstruction. Surgical repair may require resection of conal musculature.

The acute effects of an injection of contrast material (Renovist) on intraerythrocytic and extracellular pH was studied at cardiac catheterization in 72 patients with congenital heart disease. A decrease in mean extracellular (plasma) pH (7.444 plus or minus .006 to 7.419 plus or minus 0.009) and an increase in mean intraerythrocytic pH (7.204 plus or minus .005 to 7.232 plus or minus .006) were observed within minutes after injection (p smaller than .01). In 17/72 patients, simultaneous measurements of oxygen affinity for hemoglobin as characterized by P50 (oxygen tension at 50% O-2 saturation) corrected to in vivo arterial pH decreased from a mean of 26.4 to 25.2 mm Hg (p smaller than .01). It is postulated that the acute increase in intraerythrocyte pH and increased affinity of hemoglobin for oxygen are due to a decrease in intracellular hydrogen ion concentration induced by the increase in plasma osmolality with subsequent shift of hemoglobin oxygen equilibrium via the Bohr effect.

The outcome in 13 children with congenital valvar aortic stenosis who underwent a repeat valvotomy for significant residual obstruction was reviewed. There was no hospital mortality (confidence limits 0% to 32%), while one late death (8%) occurred (confidence limits 1% to 57%) following the repeat procedure. Cardiac catheterization was performed in 10 patients (77%) following the second valvotomy (2 years, 7 months mean follow-up): (1) three children, all infants at initial valvotomy, had a peak systolic ejection gradient less than or equal to 40 mm Hg and only mild, at most, aortic regurgitation after the repeat operation; (2) the other seven had a peak systolic ejection gradient greater than 40 mm Hg or at least moderate aortic regurgitation, of whom two were infants at first valvotomy while the others ranged in age from 1 to 12 years at initial surgery. These results suggest that repeat aortic valvotomy may not significantly improve the hemodynamic status of those who did not require initial valvotomy early in life.

The effect of the Mustard procedure on growth was assessed in 45 patients with simple D-transposition of the great arteries (DTGA) surviving for at least one year after operation. Growth failure (below the third percentile for height, weight, or both) was found in 25 of the 45 patients preoperatively and in 8 patients postoperatively. The principal factors associated with poor growth before repair were advancing age, increased pulmonary and systemic flow, and subpulmonic stenosis. In those patients without postoperative growth failure, growth had returned to the normal range within two years. All patients with retarded growth after the Mustard procedure had had preoperative growth failure as well. In addition, all 8 patients with postoperative growth failure had one or more major residual hemodynamic abnormalities, whereas residual lesions were present in only 10 of the 37 patients with normal postoperative growth.

A retrospective study of our experience in the placement of 19 consecutive balloon-tipped catheters in the pulmonary artery of 18 children disclosed that the procedure can be performed with relative ease in the intensive care unit without the aid of fluoroscopy. Insertion of the catheters was not associated with any serious complications. Catheter malfunction, however, occurred in 9 of 18 patients: balloon rupture in 6 and clot formation in 3. Comparison of pulmonary capillary pressure through a balloon-tipped catheter and venous pressure through a central venous line indicates that, in the absence of significant pulmonary disease requiring high positive end expiratory pressure, or significant left heart dysfunction, a central venous pressure line is frequently adequate for monitoring right heart pressures and as a guidance to fluid therapy.

To evaluate the effects of acute afterload reduction, hydralazine, 0.2 mg/kg, was administered at cardiac catheterization to seven infants who had a large ventricular septal defect (VSD). The infants were 2.5 - 11 months old (mean 5.1 months). Before and 5, 15, 25 and 35 minutes after hydralazine, aortic, pulmonary capillary wedge, pulmonary artery, right atrial and superior vena caval pressures and saturations, heart rate and oxygen consumption were measured. Hemodynamic effects were noted after 5 minutes but were most pronounced 35 minutes after hydralazine. Prehydralazine baseline data were therefore compared with values 35 minutes after hydralazine. Pulmonary flow did not changes, but systemic flow increased significantly (4.5 +/- 0.2 to 6.7 +/- 0.5 liters/min/m2 [mean +/- SEM], p less than 0.001). The pulmonary-to-systemic flow ratio decreased by 32% (3.4 +/- 0.4 to 2.3 +/- 0.2, p less than 0.001) and the absolute left-to-right shunt decreased by 24% (10.8 +/- 1.3 to 8.2 +/- 1.2 liters/min/m2, p less than 0.01). Hydralazine caused a significant decrease in systemic resistance (13.9 +/- 0.7 to 9.5 +/- 0.7 U, p less than 0.001). Pulmonary resistance, aortic, pulmonary artery and pulmonary capillary wedge pressures, heart rate and oxygen consumption did not change after hydralazine. Right atrial pressure decreased slightly (4.0 +/- 0.6 to 2.4 +/- 0.6 mm Hg, p less than 0.05). In conclusion, hydralazine caused a significant increase in systemic blood flow and a significant decrease in both pulmonary-to-systemic flow ratio and absolute left-to-right shunt in seven infants with a large VSD. These effects appear to be related to the decrease in systemic resistance that occurred with hydralazine. Although limited to the acute setting, these findings suggest that hydralazine may be beneficial in the management of infants with a large VSD.

The influence of an exercise intervention program on cardiac dimensions was studied in 79 normal children (aged 6 to 7 years) in an experimental (n = 38) and control (n = 41) group. The experimental group participated in an aerobic exercise session that met four days/week for 8 months. Anthropometric measurements and M mode echocardiograms were obtained before and after the intervention program. Comparison of the data between groups revealed no significant (probability [p] greater than 0.05) differences in left ventricular end-diastolic dimension, shortening fraction or resting heart rate. Left ventricular posterior wall thickness exhibited a significant increase (p less than 0.0256) from 3.9 to 4.7 mm in the experimental group compared with an increase from 4.3 to 4.6 mm in the control group after correcting for preintervention differences with an analysis of covariance. Likewise, left ventricular mass increased significantly (p less than 0.0004) from 21.2 to 27.4 g in the experimental group compared with 23.4 to 25.8 g in the control group. These findings indicate that when compared with control subjects, young children involved in an aerobic exercise program showed progressive increases in left ventricular posterior wall thickness and left ventricular mass and no change in left ventricular end-diastolic dimension, shortening fraction or resting heart rate.

ROWTH G DISTURBANCE is a major consequence of congenital heart disease in infancy and childhood. The severity of growth retardation is generally dependent on the anatomic lesion and its functional effects. Most children with mild defects grow normally. In addition to the symptoms and limitations imposed by the cardiovascular malformation, the child with delayed physical development may not only lag in height and weight maturation but also in motor skills and in the development of intelligence.’ The child with retarded development will not do as well in athletics or school and may be kept back a year or placed in a class more appropriate to his chronological age. Children with growth failure tend to be treated as younger than age, are teased by peers, and become socially isolated. As a consequence, they often feel rejected and may become either very passive or aggressive. The goal of surgery in infants and children with congenital heart disease is not only the saving of life but also the prevention of the adverse effects of congenital heart disease on growth, and intellectual and psychologic development. Growth assessment may provide the parents and the postoperative child with a reassurance they need about the individual’s health. The purpose of this report is to examine to what extent palliative and corrective surgery has influenced subsequent growth.