Phenotypic features and impact of beta blocker or calcium antagonist therapy on aortic lumen size in the Marfan syndrome.

Systematic, prospective data regarding phenotypic features, including echocardiographic findings, in pediatric patients with the Marfan syndrome are lacking. In addition, limited and conflicting information exists regarding the impact of pharmacologic therapy on aortic growth rate in children. Fifty-three children and adolescents with the Marfan syndrome underwent physical examination, anthropometric evaluation, and echocardiography. The relation of pharmacologic therapy to aortic growth rate was examined in the 44 subjects in whom serial echocardiograms were recorded. Although boys and girls did not differ in ocular, skeletal, or cardiovascular manifestations, aortic dilatation tended to be more common in boys (86% vs 72%). Children with aortic dilatation at baseline (42 of 53 or 79%) were more likely to also have scoliosis and mitral prolapse (both p <0.005). The medicated patients had slower aortic growth than the unmedicated patients with regard to both absolute aortic growth rate (p <0.01) and aortic growth rate adjusted for age and body size (p <0.005). Nevertheless, major cardiovascular complications developed in 5 patients despite long-term pharmacologic therapy. In conclusion, beta-blocker and calcium antagonist therapy retards aortic growth rate in children and adolescents with the Marfan syndrome.

Incidence and risk factors for mitral valve prolapse in severe adolescent idiopathic scoliosis.

Mitral valve prolapse (MVP) is known to be associated with thoracic skeletal anomalies. To determine the incidence and risk factors for mitral valve prolapse in the adolescent population with severe idiopathic scoliosis (IS), a prospective follow-up study on 139 adolescent patients with IS from the Pediatric Orthopedic Service was undertaken. Data collected included age, sex, medical and family history, physical exam, electrocardiogram and echocardiogram, spinal x-rays, and pulmonary function tests. MVP was detected by echocardiogram in 13.6% (19/139) of patients with IS as compared with 3.2% in 154 age- and weight-matched controls (p < 0.006). All patients with MVP were asymptomatic and a systolic click or murmur was detected on the single preoperative exam only in 37% (7/19) of them. Patients with MVP and IS weighed less (45.1 +/- 2.0 vs 51.8 +/- 0.1 kg, p < 0.002) as compared with those IS patients without MVP. The electrocardiogram was abnormal in 21% (4/19) of patients with MVP as compared with only 1.6% (2/120) of patients with IS but no MVP. The two groups did not differ with respect to age at diagnosis, severity of scoliosis, positive family history of scoliosis, or the presence of restrictive lung disease. Though IS was more prevalent in females (79%), the presence of MVP was not related to gender. MVP was persistent in 10 of the 19 patients reevaluated by echocardiogram 2-4 years after spinal surgery. We conclude that MVP is four times more common in patients with severe IS than in the normal adolescent population, and is associated with a lower body weight in IS patients with MVP than in IS patients without MVP. The persistent nature of MVP, even after corrective spinal surgery, may be related to factors other than geometric changes of the heart caused by abnormal thoracic curvature.

Late follow-up after venous switch operation (Mustard procedure) for simple and complex transposition of the great arteries.

Most adolescents and young adults born with complete transposition of the great arteries (TGA) and alive today are survivors of the Mustard operation. This study reports on the serial, long-term (from 10 to > 20 years) follow-up of 85 patients who underwent this operation between 1971 and 1981. Of these, 63 had simple and 22 complex TGA. The age at surgery ranged from 2 days to 17 years. The early mortality rate was 10.5% and the late mortality 9.2%. The actuarial survival rate after 15 years was 86% for simple and 64% for complex TGA. Event-free survival after 15 years was 77% for simple and 46% for complex TGA. Yearly review of electrocardiograms and, less frequently, of Holter tracings disclosed a lower mean resting heart rate and decrease over time in sinus rhythm and an increase in active arrhythmias. Fifty-two percent had resting sinus rhythm and 17% had active arrhythmias at 16 to 20 years of follow-up. Exercise stress testing in 21 patients revealed resumption of sinus rhythm during exercise but significant diminution of endurance time and peak heart rate response. Seven of the survivors (9.2%) required reoperation. Of these, 4 had severe tricuspid regurgitation following patch closure of ventricular septal defect. This study shows gratifying long-term and event-free survival for the majority of patients who underwent surgery by this venous switch procedure.

A five-year clinical experience with 112 Blalock-Taussig shunts.

In spite of recent advances in neonatal open repair for complex cyanotic heart disease, some patients require palliation with a systemic-to-pulmonary artery shunt. We report a 5-year experience (1985-1990) with 112 Blalock-Taussig shunts. Forty-six of the 92 patients had some variant of tetralogy of Fallot, with a wide spectrum of diagnoses in the remainder. The median age at surgery was 3 months. A classic Blalock-Taussig shunt was done in 26% (group I), and a 4- or 5-mm PTFE graft was utilized in the remainder (group II). The technical aspects of each of the procedures are reviewed. There were three early deaths in the entire group, none of them related to Blalock-Taussig shunt function. There was no incidence of early shunt insufficiency, bleeding, infection, limb ischemia, or pulmonary artery distortion. There was a 21% incidence of clinical congestive heart failure, seen somewhat more commonly in group I. The overall need for reshunting/open repair was similar in both groups, but there was a statistically longer interval between the initial Blalock-Taussig shunt and the second procedure in group I (21.6 vs 12.4 months). The Blalock-Taussig shunt remains a safe, reliable, and effective means of increasing pulmonary flow.

Cardiac issues in the pediatric emergency room.

A great variety of problems referable to the cardiovascular system can prompt a visit to the pediatric emergency room. From the initial presentation of congenital heart disease, to the subsequent life-long management of these patients, to miscellaneous problems like Kawasaki disease and chest pain, the front-line pediatrician must be skilled in the recognition and early management of myriad complaints. This article focuses on information that can assist the emergency pediatrician in the evaluation and treatment of the cardiac patient from arrival in the emergency room until transfer of care to the pediatric cardiologist or inpatient staff.

Congenital aortic regurgitation: natural history and management.

OBJECTIVES AND BACKGROUND: Congenital aortic regurgitation is rare as an isolated lesion. We describe seven children with no physical features of the Marfan syndrome in the patients or their families and no other cardiac lesions who had congenital valvular aortic regurgitation. METHODS: From 1954 to the present, seven children with auscultatory and physiologic characteristics of aortic regurgitation were evaluated for a total of 108 patient-years. We report on their natural history, clinical and laboratory findings, management and outcome. RESULTS: In five of the seven children congenital aortic regurgitation was diagnosed in infancy. In four, progressive severity of the regurgitation led to valve replacement at age 3, 10, 15 and 20 years, respectively, and to resection of an aneurysm of the ascending aorta in the 10-year old patient. Two patients had cystic medial necrosis on aortic biopsy. One of these patients died after reoperation for dissecting aneurysm of the thoracic aorta at 22 years of age; the other died after dissection and rupture of the ascending aorta at age 25 years. After obstructing pannus developed, the 3-year old patient underwent replacement of the St. Jude valve at age 10 years. The other three patients were asymptomatic at last follow-up at age 8, 10 and 20 years, respectively. CONCLUSIONS: Supportive management is recommended until it becomes necessary to intervene surgically when regurgitation becomes severe. The need for surgical treatment is indicated by the appearance of a diastolic thrill, left ventricular strain on the electrocardiogram or other evidence of left ventricular dysfunction on the echocardiogram or exercise stress testing by treadmill or radionuclide cineangiocardiography. Close follow-up of these patients is important to detect progression of aortic regurgitation, especially in the presence of cystic medial necrosis.

MR imaging of cardiac mass in Wegener granulomatosis.

Wegener granulomatosis is a disorder of unknown etiology characterized by a necrotizing granulomatous vasculitis that primarily affects the paranasal sinuses, lungs, and kidneys. It is usually a disease of adults with infrequent cardiac involvement. We report the unusual presentation of this disorder in an adolescent with a cardiac mass. The findings on two-dimensional echocardiography and MR imaging are discussed.

Prolonged survival in patients with beta-thalassemia major treated with deferoxamine.

To determine whether survival of patients with beta-thalassemia major has been prolonged by management that utilizes hypertransfusion and chelation with deferoxamine, we analyzed longevity by the Kaplan-Meier product-limit method. Group 1 patients (n = 71) followed between 1960 and 1976 with a low-transfusion regimen (pretransfusion hemoglobin level 7 to 8 gm/dl) and no chelation had an estimated median age of survival of 17.4 years, whereas it was 31.0 years for group 2 subjects (n = 80), who began hypertransfusion between 1976 and 1978 (pretransfusion hemoglobin level 10.5 to 11.5 gm/dl) and chelation with deferoxamine (20 to 60 mg/kg per day) (p less than 0.0001). For 70 patients who were treated with hypertransfusion and deferoxamine, we had data to calculate the ratio of total milligrams of transfusional iron to cumulative grams of deferoxamine. The 24 patients who died had a total iron burden of greater than 1.05 gm/kg; the ratio for them exceeded 31. These patients were characterized by poor compliance with chelation or by late start of therapy, with inability to receive enough deferoxamine before death. Death was preceded by arrhythmia requiring therapy in all but one, and by cardiac failure in all. Of 41 similarly iron-loaded survivors, 33 had a ratio of less than 31; only three had an arrhythmia, and five had cardiac failure. We conclude that treatment with deferoxamine, when used in amounts proportional to iron burden, delayed cardiac complications and improved longevity.

Epidemiologic aspects of Kawasaki disease in a Manhattan hospital.

Epidemiologic and clinical features of Kawasaki disease in 106 patients seen between 1980 and 1986 at The New York Hospital in midtown Manhattan were compared with those in large series from the United States, Canada, and Japan. Dissimilarities in our Kawasaki disease experience included ethnic heterogeneity of our patients (50% white, 18% black, 16% Hispanic, and 16% Oriental) and, in comparison with the Japanese experience, an older mean age (3 1/2 vs 1 1/2 years) with fewer children less than 2 years of age (32% vs 50% to 60%). In comparison with the general population of the geographic urban and suburban referral area for our hospital and in comparison with our general pediatric population, Oriental children with Kawasaki disease were overrepresented (16% vs 2%). More families of children with Kawasaki disease were members of the upper and middle class (73%) than were the population seen in general pediatrics (31.7%) at our hospital. Personal interviews with 63 families of children with Kawasaki disease and 63 control families with children paired for ethnic group, sex, and age revealed no epidemiologic differences except for use of rug shampoo within 1 month of onset in 16 episodes in 15 children with Kawasaki disease in 14 families (22% of families) compared with two families of control children (3%) (P less than .001).

Correlation of electrocardiographic and echocardiographic changes in Kawasaki syndrome.

To determine whether changes in ECGs correlated with abnormalities in echocardiograms in children with acute and convalescent Kawasaki syndrome, we undertook a retrospective analysis of 44 patients examined during the first 2 weeks of illness and followed for at least 6 months, 31 for longer than 1 year. We analyzed 360 ECGs, 282 echocardiograms, and clinical status. Results of echocardiography showed 18 children with myocardial dysfunction, pericardial effusion, and/or coronary arterial abnormalities (41%); 26 children had no abnormalities. All 18 of the former and all but 10 of the latter had abnormalities on serial ECGs that normalized on recovery. Thirty-four of 44 patients (77%) had ECG abnormalities. These abnormalities were most prevalent in the first month: 68% appeared in the first week, about 50% in the second to fourth weeks, 16% at 2 months, and 10% at 3 months. The type of ECG change did not predict the type of echocardiographic abnormality, except that low voltage of QRS was found only with pericardial effusion. The most frequent early changes were in T waves, which were flattened in 28 (64%). Later 12 patients had high, peaked, abnormal T waves. Prolonged PR interval occurred in 13 patients, seven of whom had echocardiographic abnormalities. Of six with prolonged QT interval, four had abnormal echocardiograms. The possibility of having an abnormal echocardiogram increased with the number of ECG changes: from 0 risk with no change to 37% for one, 47% for two, 80% for three, and 100% for four changes in serial ECGs. Both ECGs and echocardiograms are important in early and subsequent evaluation of these patients. Inasmuch as the ECGs showed abnormalities in 77% whereas echocardiograms showed changes in 41%, we believe that serial ECGs in comparison with prior tracings offer the more sensitive noninvasive indicator of pancarditis in young children during acute and convalescent Kawasaki syndrome.

Coronary artery involvement in Kawasaki syndrome in Manhattan, New York: risk factors and role of aspirin.

Since January 1980, 110 children having 113 attacks of Kawasaki syndrome were studied. Age at onset was 7 weeks to 12 years (mean 3 6/12 years, median 2 9/12 years); 77% were younger than 5 years of age; the male to female ratio was 1.8; racial distribution was 52% white, 19% black, 14% Hispanic, and 16% Asian. Protocol of management consisted of high-dose aspirin (100 mg/kg/d) until afebrile, and then 81 mg every day until free of coronary aneurysm. Two-dimensional echocardiograms were done weekly during the acute stage, at 2 and 6 months after onset, and yearly if a coronary abnormality was detected. At 1 month, 51 coronary arterial abnormalities were present in 25 patients. Risk factors for a coronary abnormality were duration of fever greater than or equal to 2 weeks, level of platelet count, marked elevation of ESR, and age younger than 5 years. No statistically significant difference in incidence of aneurysms was detected between patients on high-dose aspirin and those on medium-or low-dose aspirin.

Sympathetic nervous system and exercise tolerance response in normotensive and hypertensive adolescents.

Comparative evaluation of isometric and dynamic exercise performance in normotensive, borderline hypertensive and hypertensive adolescents was made. Hemodynamic changes were correlated with level of adrenergic sympathetic nervous system activity as measured by plasma epinephrine and norepinephrine values. No significant intergroup differences were found with respect to isometric exercise with the exception of high peak isometric exercise heart rates and plasma epinephrine levels in the patients with significant hypertension. During dynamic treadmill testing, patients with the most marked hypertension demonstrated a statistically significantly greater tachycardia response to exercise that correlated with highest peak exercise epinephrine levels. The most hypertensive systolic dynamic exercise response was evidenced in the borderline hypertensive group in which peak exercise norepinephrine values were significantly higher than in other groups. Maximal exercise serum lactate levels were higher in hypertensive patient groups than in normotensive subjects. Altered hemodynamic response to peak dynamic exercise appears to exist in adolescents with borderline and significant hypertension and is in part mediated by altered activity of the sympathetic nervous system.

Noninvasive diagnosis of persistent fetal circulation versus congenital cardiovascular defects.

Congenital cardiovascular anomalies associated with right-left atrial or ductal shunts must be excluded before a diagnosis of persistent fetal circulation (PFC) can be made. Despite the advent of 2-dimensional echocardiography (2-D echo), this differentiation can be difficult and may require cardiac catheterization with selective angiography. Fifteen consecutive cases were analyzed in which difficulty was encountered with this differential diagnosis, and experience with the use of cardiac auscultation, the 12-lead electrocardiogram (ECG), arterial blood gas determinations and 2-D echo, both alone and with injection of venous contrast material, is reviewed. Electrocardiographic abnormalities of ventricular axis, hypertrophy or dominance (p = 0.002) and suspicion of cardiovascular disease on 2-D echo (p = 0.011) were the most useful findings in differentiating patients with PFC from those with congenital cardiovascular abnormalities. The ECG was the most sensitive test (100% sensitivity, 90% specificity), while 2-D echo was the most specific (100% specificity, 75% sensitivity). Evidence of right-left shunting at atrial or ductal levels or both did not differentiate between the groups; both groups had evidence of such shunts. A decision tree was developed to facilitate this differential diagnosis, which uses the ECG and 2-D echo. If the ECG reveals no abnormalities of ventricular axis, dominance or hypertrophy, the 2-D echo shows no structural abnormalities, and total anomalous pulmonary venous return and coarctation/interruption of the aorta are specifically excluded, a congenital cardiovascular anomaly is effectively eliminated. We suggest that this approach can optimize the management of the cyanotic newborn with suspected PFC by eliminating the risks of cardiac catheterization and angiography without missing the diagnosis of a major structural cardiovascular anomaly.