Prospective identification of anomalous origin of left coronary artery from the right sinus of valsalva using transthoracic echocardiography: importance of color Doppler flow mapping.

Four cases are discussed in which anomalous origin of the left coronary artery from the right sinus of Valsalva was identified prospectively by transthoracic echocardiography when the anomalous coronary passed between the great arteries. Color Doppler flow mapping was a critical component in identifying the anomalous coronary, particularly when the proximal course of the left main coronary was intramural.

Factors affecting longevity of homograft valves used in right ventricular outflow tract reconstruction for congenital heart disease.

BACKGROUND: Few studies have explored the long-term function of cryopreserved homograft valves used for reconstruction of the right ventricular tract (RVOT) in patients with congenital heart disease. METHODS AND RESULTS: Among 205 patients receiving cryopreserved homografts for reconstruction of the RVOT between November 1985 and April 1999, the outcome of 220 homografts in 183 operative survivors was analyzed. There were 150 pulmonary and 70 aortic homografts used. Median age at implantation was 4.4 years (mean 6.9+/-7.6 years, range 3 days to 48 years). End points included (1) patient survival, (2) homograft failure (valve explant or late death), and (3) homograft dysfunction (homograft insufficiency or homograft stenosis). Survival was 88% at 10 years. Freedom from homograft failure was 74+/-4% at 5 years and 54+/-7% at 10 years. Univariable analysis identified younger age, longer donor warm ischemic time, valve Z: value <2, and previous procedure as risk factors for homograft failure and dysfunction. Aortic homograft type and extracardiac operative technique predicted homograft failure but not dysfunction. For patients

Evaluation of the pediatric patient with a cardiac murmur.

In conclusion, the evaluation of cardiac murmurs represents one of the most skilled and demanding aspects of the pediatric physical assessment. It does provide a significant service to patients and a level of satisfaction for competent practitioners, and referral is not always warranted. The decision for referral is based on the presumed diagnosis, confidence of the examiner, and level of parental anxiety. Most often, referral to a cardiologist for directed investigation rather than direct echocardiographic assessment serves patients best.

Advances in the care of children with heart disease.

As we enter the next millennium, we are encouraged by the progress that has been made in the care of neonates, infants, and children with heart disease. Surgical repair can be offered at an earlier age with excellent results. Diseases that were uniformly fatal in the past have improved outcomes. Research continues in the area of interventional devices such that surgical repair might be eliminated or delayed. We continue to look forward to advances in the next several years that will allow for future improvement in outcome, better quality-of-life and better long-term results.

The cardiac murmur. When to refer?

This review article outlines the significance and defines the skills important for the diagnosis and recognition of common innocent cardiac murmurs in childhood. The origin of heart sounds and murmurs is reviewed, and an approach to pediatric murmur evaluation is presented. The seven innocent murmurs of childhood and adolescence are reviewed in detail. Further diagnostic evaluation and referral is dependent on the comfort factor and experience in recognition and correct characterization of these murmurs.

Verapamil sensitive incessant ventricular tachycardia in the newborn.

Incessant VTs in infancy are often poorly tolerated. Response to conventional antiarrhythmic therapy is disappointing. The present case involves a 3-week-old infant with an incessant VT of a left bundle branch block, and normal axis morphology. This tachycardia was inducible with both atrial and ventricular pacing, but not by premature stimulation. Tachycardia persisted despite treatment with adenosine, esmolol, procainamide, and flecainide. Intravenous verapamil suppressed tachycardia and prevented inducibility, and no further recurrences were seen on oral verapamil. This case suggests that some incessant VTs in infancy may be due to calcium channel related afterdepolarizations or triggered activation.

Aprotinin improves outcome of single-ventricle palliation.

BACKGROUND: Elevation of pulmonary vascular resistance as a consequence of cardiopulmonary bypass may lead to failure of single-ventricle palliation. We reviewed our experience with aprotinin, a nonspecific serine protease inhibitor, to determine whether it could ameliorate the inflammatory effects of cardiopulmonary bypass and improve outcome of single-ventricle palliation. METHODS: Forty-six consecutive patients undergoing single-ventricle palliation using cardiopulmonary bypass were reviewed retrospectively. Aprotinin was used in 8 of 30 bidirectional cavopulmonary shunt and 10 of 16 Fontan procedures. RESULTS: Aprotinin use was associated with a decrease in the early postoperative transpulmonary gradient among patients undergoing Fontan and bidirectional cavopulmonary shunt procedures. The bidirectional cavopulmonary shunt aprotinin group had a higher oxygen saturation and a decrease in quantity and duration of thoracic drainage. Among patients receiving aprotinin there were no episodes of mediastinitis, thrombus formation, or renal failure. CONCLUSIONS: Aprotinin use in single-ventricle palliation was associated with decreased transpulmonary gradient and increased oxygen saturation consistent with decreased pulmonary vascular resistance. This retrospective study suggests that aprotinin has a favorable impact on the early postoperative course of single-ventricle palliation.

Twenty-year experience with repair of complete atrioventricular septal defects.

BACKGROUND: To determine factors predicting mortality and morbidity after repair of complete atrioventricular septal defect, we retrospectively analyzed preoperative, operative, and postrepair factors on the outcome of 115 consecutive complete atrioventricular septal defect repairs at The Children's Hospital of Wisconsin between January 1974 and December 1993. METHODS: For the entire experience the operative mortality was 13.9% (16 patients). During the most recent era, January 1988 to December 1993, operative mortality was 3.6% (2 of 55 patients). This was significantly improved from the two previous eras, January 1974 to December 1980, 28% (7 of 25) and January 1981 to December 1987, 20% (7 of 35 patients) (p = 0.02). There were seven late deaths; 10-year actuarial survival, including operative mortality was 81%. Age at complete repair decreased; before 1982 all patients were more than 12 months of age, whereas after 1982 64% (56 of 88 patients) were 12 months of age or less. RESULTS: Moderate or severe preoperative left atrioventricular valve regurgitation was not a risk factor for operative mortality. For operative survivors with moderate to severe preoperative left atrioventricular valve regurgitation (n = 17), late postoperative left atrioventricular valve regurgitation (follow-up data available on 15 patients) was significantly reduced (severe = 1, moderate = 5, mild = 9; p = 0.007). CONCLUSIONS: Early mortality was predicted by the era of surgical repair. Conversion to routine repair during infancy was achieved with a simultaneous decrease in operative mortality. For patients with moderate to severe preoperative left atrioventricular valve regurgitation, significant improvement in the degree of left atrioventricular valve regurgitation can be expected without an increase in operative or late mortality or morbidity.

Does an additional source of pulmonary blood flow alter outcome after a bidirectional cavopulmonary shunt?

BACKGROUND: The bidirectional cavopulmonary shunt has become an important intermediate step in the treatment of pediatric patients with single ventricle physiology who are ultimately destined for palliative surgery. We wanted to know whether there would be risks or benefits if an additional source of pulmonary blood flow was left after a bidirectional cavopulmonary shunt. METHODS AND RESULTS: We retrospectively reviewed the medical and surgical records of all patients who underwent a bidirectional cavopulmonary shunt at the Children's Hospital of Wisconsin between January 1991 and December 1993. A total of 43 patients were identified. Anatomic diagnoses included double inlet left ventricle (14 patients), tricuspid atresia (8 patients), pulmonary atresia with intact septum (6 patients), single right ventricle (5 patients), hypoplastic left heart (3 patients), unbalanced atrioventricular septal defect (3 patients), and other complex lesions (4 patients). We then divided the patients into two groups for purposes of analysis. Group 1 had only the cavopulmonary shunt as a source of pulmonary flow (22 patients); group 2 had an additional source of pulmonary flow (21 patients). Patient age at the time of cavopulmonary shunt ranged from 6 months to 12 years, with group 1 patients being younger (31 versus 45 months, P = .05). Group 2 patients had higher postoperative central venous pressures (17.8 versus 14.1 mm Hg, P < .001) and oxygen saturations (86% versus 81%, P < .001) than did group 1 patients. There was no statistical difference between groups in the number of chest tube days or hospital days. There was 1 early death in group 1 related to severe ventricular dysfunction and 1 late death in group 2 related to sepsis. Five patients in group 2 were readmitted to the hospital for drainage of a large chylothorax compared with none in group 1 (P < .02). CONCLUSIONS: We conclude that patients with an additional source of pulmonary blood flow after bidirectional cavopulmonary shunt have higher postoperative central venous pressures, have higher oxygen saturations, and are at risk for the late development of a chylothorax.

A comparison of iohexol and diatrizoate-meglumine in children undergoing cardiac catheterization.

Iohexol (Omnipaque) and meglumine and sodium diatrizoate (Renografin-76) were compared in a double-blind, randomized study for their efficacy, safety, and hemodynamic effects as angiographic contrast agents in children. Forty-four children were randomly allocated to receive either iohexol or diatrizoate as a component of their routine or emergency cardiovascular evaluation. Following age stratification, baseline physiologic parameters were not significantly different between patients receiving either iohexol or diatrizoate. After systemic ventricular injection, iohexol produced significantly less hemodynamic alteration in systemic systolic blood pressure, systemic ventricular end-diastolic pressure, and dP/dt. Less alteration in heart rate and significantly less effect on the QT interval were seen with iohexol. Image quality was comparable, although significantly more patient mobility was associated with diatrizoate-meglumine. This study shows that iohexol, a nonionic contrast medium, causes less hemodynamic disturbance than diatrizoate-meglumine in children. Therefore, its use to be preferred in these potentially high-risk patients.

Interventricular septal motion and left ventricular function in patients with atrial septal defect.

In order to assess whether the paradoxical motion of the interventricular septum seen in patients with atrial septal defect (ASD) is due to a true abnormality in septal contraction, eight patients with ASD (age, 1.6-17 years) and eight age-matched control patients were studied using qualitative and quantitative two-dimensional (2D) and M-mode echocardiography. 2-D-echocardiographic images recorded from the parasternal short-axis projection at the level of the papillary muscles and 2D-directed M-mode tracings at this level were obtained. Comprehensive wall motion analysis of the left ventricular (LV) endocardial and epicardial borders was performed using both fixed reference and center of mass (floating reference) models. Our results indicate that interventricular septal wall motion and function are normal in patients with ASD. The apparent "paradoxical" motion is due to excessive anterior motion of the entire left ventricle, and is present only when a fixed reference system is used to assess myocardial motion, but is not present when a center of mass (floating reference system) is employed. Left ventricular function assessed by % area and perimeter change, mean radial shortening fraction, and mean radial wall thickening (2D) as well as LV shortening fraction and septal and posterior wall thickening (M-mode) was not significantly different between the two groups. Standard M-mode tracings can therefore be used to assess LV function despite this apparent abnormal septal motion.

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 6.5 mm), and failure to achieve an adequate aortic orifice (greater than 6 mm), at operation were identified as factors associated with increased mortality. Initial perioperative survival was better with closed aortic valvotomy. However, there was no significant difference in overall operative survival between closed (9/18, 50%) and open (8/22, 36%) aortic valvotomy (p = 0.26). The incidence of early reoperation (less than 1 year of age) was greater in perioperative survivors undergoing closed valvotomy (7/13, 54%) rather than open valvotomy (1/10, 10%) (p less than 0.05). In conclusion, long-term survival among patients with critical neonatal aortic valve stenosis remains disturbingly low (13/40, 32%) and has not significantly improved over the past 20 years.

Postoperative hypertension after repair of coarctation of aorta in children: protective effect of propranolol?

The effects of the nonselective beta blocker, propranolol, on coarctectomy-induced hypertension were evaluated relative to changes in cardiac function, sympathetic tone, and plasma renin activity (PRA). A randomized, placebo-controlled, double-blind, age-stratified design was employed. Propranolol (n = 11, mean age 9.4 years) or placebo (n = 12, mean age 10.2 years) was started 2 days before surgery and continued until 6 days after surgery. In patients on placebo, systolic and diastolic blood pressure, heart rate, and cardiac index increased rapidly and peaked within 1 day after surgery. Over the ensuing days these increases gradually abated. Plasma epinephrine and PRA increased markedly within 12 hours after surgery, but returned quickly toward preoperative levels. In contrast, plasma norepinephrine increased more gradually and remained elevated longer. In patients on propranolol, systolic blood pressure, heart rate, cardiac index and PRA showed only negligible increases. However, diastolic blood pressure, increased even faster with propranolol than with placebo but to the same extent. Plasma catecholamines showed similar increases on the two treatments. Only in the placebo group was the code broken in the intensive care unit because of hypertension uncontrolled by sodium nitroprusside; this occurred in 6 of 12 patients. We conclude that nonselective beta blockade ameliorates the hyperdynamic circulation and increase in systolic blood pressure following coarctectomy. The initial increase in diastolic blood pressure following surgery in the propranolol group may have been caused by vascular beta-2-receptor blockade resulting in unopposed alpha-receptor-mediated vasoconstriction.

Exercise in children before and after coarctectomy: hemodynamic, echocardiographic, and biochemical assessment.

After repair of coarctation of the aorta, some patients with normal blood pressure at rest have an exaggerated hypertensive response to activity. Blood pressure response to exercise was studied in 15 children, aged 5 to 15 years, prior to and at periods up to 6 months following coarctectomy. Preoperatively, 11 of 15 children had systolic hypertension at rest and 12 of 15 after exercise. After surgery, only one child had mild systolic hypertension at rest, whereas exercise-induced hypertension persisted in 33% of patients (all older than 10 years). Exercise plasma renin activity was elevated preoperatively but normalized following surgery. No significant difference was seen in resting and exercise plasma catecholamine levels measured before and after surgery. Over the follow-up period of 6 months, echocardiographic evidence of left ventricular hypertrophy regressed in the younger patients but not in the older patients with exercise-induced hypertension. Exercise testing defines a subgroup of patients with exercise-induced hypertension evident soon after surgery. Structural upper segment arterial vessel wall changes in the older patient may explain these observations.

Evaluation of pulmonary vascular resistance in the presence of a large pulmonary arteriovenous malformation.

A 1 day old infant presented with severe cyanosis and congestive heart failure. Cardiac catheterization confirmed the clinical suspicion of a pulmonary arteriovenous malformation of the right lower lobe. Catheter occlusion of the right lower pulmonary artery allowed evaluation of the integrity of the remaining pulmonary vascular bed as well as improvement of the patient's hemodynamic condition pending more definitive therapy.