Hypertension in a cohort of obese Caucasian children and adolescents and its association with glycometabolic indices: A proposed screening tool.

BACKGROUND AND AIM: Hypertension (HTN) is common among obese children and adolescents and increases their cardiovascular risk later in adulthood. The aim of the study was to evaluate the prevalence of HTN identified by office blood pressure (BP) measurement and ambulatory BP monitoring (ABPM) in a cohort of obese children and adolescents and its association with anthropometric and glycometabolic indices. METHODS AND RESULTS: Seventy consecutive obese Caucasian children and adolescents aged 7-16 years were enrolled. Patients underwent ABPM, echocardiogram and carotid ultrasonography. Sex- and age-adjusted logistic multivariable analysis models were used to assess the association between HOMA-IR, HOMA-ß, QUICKI with HTN at ABPM. Receiver Operation Curve (ROC) analysis with Youden J statistics was used to identify the optimal HOMA-IR, HOMA-ß and QUICKI cut-off to predict HTN at ABPM. Hypertensive office BP was found in 25.7% of obese patients. ABPM diagnosed HTN in 34.9% of patients: 20.6% of obese patients had masked HTN (MHTN), and 12.7% had white coat HTN (WCH). Hypertensive obese patients (according to ABPM) had higher HOMA-IR and HOMA-ß, and a lower QUICKI than normotensive subjects. HOMA-IR, HOMA-ß and QUICKI predicted HTN at ABPM in obese patients in age- and sex-adjusted logistic multivariable models. Optimal cut-offs to predict HTN at ABPM in obese patients were: HOMA-IR ≥ 3.30, HOMA-ß ≥ 226.7 and QUICKI <0.33, with high sensitivity. CONCLUSIONS: A sequential testing strategy applying office BP and glycometabolic indices can identify hypertensive obese pediatric patients with high diagnostic accuracy and potentially reducing costs. This strategy needs validation in an external and larger cohort.

Tumor lysis syndrome after propranolol therapy in ulcerative infantile hemangioma: rare complication or incidental finding?

A 33-day-old female with an ulcerated infantile hemangioma (IH) undergoing oral therapy with propranolol 2 mg/kg per day developed hyperkalemia and hyperphosphatemia 24 h after starting medication. No electrocardiographic or clinical abnormalities secondary to the electrolyte changes were noticed. A laboratory tumor lysis syndrome (TLS) was diagnosed after excluding other causes of electrolyte imbalance in the diagnostic workup. No treatment was required to reverse the TLS condition, and the propranolol therapy was continued as the electrolyte alterations were only mild. One month later, the IH was remarkably reduced in size and no longer ulcerated. Maintenance of propranolol was extended for a total of 6 months. Parallel to the gradual involution of the IH, serum potassium and phosphorus levels returned within normal levels. We suggest that TLS may be a rare complication of ulcerated IH treated with propranolol. Clinicians must be aware and order appropriate screening tests for TLS in patients at risk.

Mechanical valves in the pulmonary position: a reappraisal.

OBJECTIVES: To evaluate midterm results of mechanical valves in pulmonary position in patients with pulmonary regurgitation and right ventricular dysfunction as an alternative to bioprostheses. PATIENTS: Mechanical valves (six tilting disc valves and two bileaflet valves) were implanted in eight patients previously operated on for tetralogy of Fallot (n = 7) and truncus arteriosus (n = 1), with severe right ventricular dysfunction caused by massive pulmonary regurgitation. RESULTS: All patients survived prosthesis implantation and are currently well. At follow-up (3 months to 9 years), they do not show signs of valve failure, and right ventricular function has dramatically improved in all but one, who still shows moderate ventricular hypokinesia. CONCLUSION: After operative correction of congenital heart defects in selected patients who show severe dysfunction of the right ventricle caused by pulmonary regurgitation/stenosis, mechanical valves may represent an alternative to bioprosthetic valves. The selection of the valve type is still a matter of debate. However, according to literature data, complications seem to have occurred only in patients with bileaflet mechanical valves in the pulmonary position, whereas no thromboembolic episodes or valve failure is reported in subjects with tilting disc valves in the right ventricular outflow. Tilting disc valves might perform better in the right ventricular outflow than bileaflet valves.

Balloon dilation of critical valvar pulmonary stenosis in the first month of life.

Between 1985 and 1992, 36 consecutive neonates, aged 1-29 days, weight 2.4-5.0 kg, with critical valvar pulmonary stenosis underwent attempted balloon dilation (BD). At catheterization, 30 were on prostaglandin (PGE1) therapy and 20 were intubated. The valve was successfully crossed and dilated in 34/36 (94%), including three with an echocardiographic diagnosis of valvar pulmonary atresia and a right ventricle of adequate size. The valve was first dilated with a 2- to 5-mm balloon and then with serially larger ones (up to 12 mm) to a final balloon/annulus value of 126%. The RV/systemic pressure value fell from 150 +/- 32 to 83 +/- 30%, O2 saturation rose from 91 +/- 6% to 96 +/- 4%, and PGE1 was discontinued at the end of the procedure. There were 11 complications (31%) including one early death from sepsis and necrotizing enterocolitis, endocarditis in another, two myocardial perforations, one femoral-iliac vein tear, and one transient pulse loss. A repeat BD was carried out in five patients, two of whom subsequently had surgery. At follow-up (33 +/- 23 months), the 31 patients managed by BD alone were well and had echocardiographic gradients of < 30 mm Hg in 90% and pulmonary regurgitation, considered mild in most, in 52%. In neonates with critical valvar pulmonary stenosis, we believe BD mortality is less than with surgery and is the treatment of choice.

Changing role of non-invasive investigation in the preoperative assessment of congenital heart disease: a nine year experience.

The total surgical experience of a supraregional paediatric cardiology unit over a nine year period (January 1980 to December 1988) was reviewed to assess the effect of the introduction of the full range of ultrasound techniques. A total of 1517 patients underwent cardiac surgery (955 cardiopulmonary bypass, 562 closed procedures). Of these, 485 patients (32%) did not undergo cardiac catheterisation before operation: 217 bypass (23% of all procedures under cardiopulmonary bypass) and 268 closed procedures (48%). The overall ratio of catheterisations to operations for patients undergoing palliative or corrective surgery fell from 0.97 (1980) to 0.38 (1988). The patients were classified as (a) neonates (0-28 days), (b) infants (one to 12 months), and (c) children (one to 14 years). The main impact of non-invasive surgical referral was in neonates (total catheter:operation ratio 0.38; neonates 0.2 for 1988). The surgical population was further divided according to the principal echocardiographic technique available: (a) 1980-4 cross sectional imaging; (b) 1985-6; imaging plus spectral Doppler ultrasound; (c) 1987-8; imaging plus spectral Doppler ultrasound and colour flow mapping. A fall in the catheter:operation ratio for all age groups was most pronounced in the last four years. This reflects increased familiarity and surgical confidence with non-invasive diagnostic assessment. The introduction of each new echocardiographic technique was associated with a significant fall in the total catheter:operation ratio compared with the preceding period. Six incorrect ultrasound diagnoses were made during the entire period; one of these patients died in the early postoperative period. The integration of Doppler ultrasound with cross sectional imaging has made non-invasive assessment an increasingly practical alternative to preoperative cardiac catheterization.

Anatomically corrected malposition of the great arteries: diagnostic difficulties and surgical repair of associated lesions.

Four patients, aged 5 to 14 years, had repair of abnormalities associated with anatomically "corrected malposition," a condition associated with mitral/aortic discontinuity with atrioventricular and ventriculoarterial concordance, and with the aorta lying anterior and to the left of the pulmonary artery. All 4 patients had ventricular septal defect; in addition, 1 patient had coarctation, 2 patients had pulmonary infundibular stenosis, 1 patient had acquired pulmonary valve atresia, 1 patient had acquired fixed subaortic stenosis and 2 patients had left juxtaposition of the atrial appendages. The position of the great arteries suggest corrected transposition, but the true diagnosis is made from finding atrioventricular and ventriculoarterial concordance with wide mitral/aortic separation on the cross-sectional echocardiogram. The electrocardiogram shows normal ventricular activation. Three of the 4 patients had anticlockwise rotation of the heart, which displaced the apex. This made echocardiography difficult and caused a problem in visualizing the right-sided valved conduit and closing the VSD in conventional ways. One patient died at reoperation for an obstructed conduit. Mitral regurgitation developed after resection of subaortic stenosis in 1 patient. No arrhythmias occurred in the 3 surviving patients, followed for 1 to 5 years, but the risk of subaortic stenosis remains.

Pulmonary valve agenesis and supravalvular pulmonary stenosis. Report of a case and review of literature.

Pulmonary valve agenesis is a rare malformation, and is usually associated with a ventricular septal defect. Occasionally it may be associated also with right ventricular outflow obstruction. Children with this syndrome usually die early in infancy either of intractable heart failure or of severe respiratory distress. Rare instances of association with peripheral pulmonary stenoses have also been reported. We report the case of a three-year-old girl, asymptomatic except for failure to thrive, with pulmonary valve agenesis and coarctation of the pulmonary artery trunk.

The meaning of bites on the vectorcardiogram: study in adriamycin cardiomyopathy.

We examined 106 VCGs of cancer patients receiving Adriamycin (ADM) to investigate the nature and meaning of bites. It is known that ADM causes patchy areas of myocardial degeneration and necrosis, the appearance of which is dose dependent. Thirty-eight cancer patients not yet receiving ADM were examined to provide controls. Patients were divided into six groups, according to total cumulative ADM dose received. The incidence of bites in the different groups was shown to be as follows: group 1 (controls, 38 patients) 13.1%; group 2 (1-100 mg/m2 of body surface ADM, 23 patients) 39.1%; group 3 (101-200 mg/m2, 33 patients) 42.2%; group 4 (201-300 mg/m2, 25 patients) 56%; group 5 (301-400 mg/m2, 15 patients) 66.6%; group 6 (greater than 400 mg/m2, 10 patients) 90%; groups 2-6 52.8%. These percentages show a trend which is highly correlated with the total cumulative dose of ADM (p=0.00005). The influence of age on the appearance of bites has been excluded by analyses of trends in patients below and over 50 years (p=0.08). In 80.35% of cases, bites appear on more than one plane and mostly close to the maximum vector (Vmax). We therefore think that these results favor the hypothesis that bites are the electrophysiologic expression of lesions, such as small fibrotic and necrotic areas, which interfere with the normal spread of the ventricular depolarization wavefront.