Early left ventricular abnormality/dysfunction in obese children affected by NAFLD.

BACKGROUND AND AIMS: Although it is generally accepted that non alcoholic fatty liver disease (NAFLD) is linked to increased risk of cardiovascular disease, the presence of abnormalities in cardiac function among NAFLD children is limited and controversial. Aim of the study was to detect cardiac abnormalities/dysfunction in a paediatric population of NAFLD. METHODS AND RESULTS: Anthropometric, laboratory, cardiovascular fitness, 24 h blood pressure monitoring and Doppler echocardiography parameters were obtained in 50 untreated children (37 males; mean age 12.2 + 2.5) with biopsy-proven NAFLD. Abnormalities in both cardiac function and geometry could be identified in the whole study population: prevalence of about 35% in left ventricular hypertrophy, 14% of concentric remodelling and 16% of left atrial dilatation. Furthermore children with NAFLD (NAS score <5) showed lower cardiac alterations compared to NASH patients (NAS score >5). After adjusting for age, sex and BMI, a positive correlation was found only between LV mass and NAS score (p < 0.001). CONCLUSION: Our results suggest that cardiac dysfunction can be detectable early in NAFLD children and this is not linked to cardiovascular and metabolic alteration, other than to liver damage. Although as a preliminary stage, we can speculate a possible direct relationship between liver and heart steatosis, already occurring during childhood.

Contrast echocardiography for pulmonary arteriovenous malformations screening: does any bubble matter?

AIMS: To evaluate diagnostic accuracy of contrast echocardiography (CE) as compared with CT, for the screening of pulmonary arteriovenous malformations (PAVMs) in hereditary haemorrhagic telangiectasia (HHT); to evaluate the clinical significance of semi-quantitative analysis of a shunt on CE. METHODS AND RESULTS: A blinded prospective study was conducted in 190 consecutive subjects at risk of HHT who underwent screening for PAVMs, including clinical evaluation, pulse oximetry, standard and CE, and chest multirow CT without contrast medium. A semi-quantitative analysis of the shunt size was performed according to the contrast echo opacification of the left-sided chambers: Grade 0, no bubbles; 1, occasional filling with <20 bubbles; 2, moderate filling; 3, complete opacification. The first 100 patients were compared with 100 controls. A total of 119 (63%) patients had positive CE (32.2% Grade 1, 13.1% Grade 2, 11% Grade 3, 6.3% with patent foramen ovale). The overall diagnostic performance of CE was sensitivity 1.00, specificity 0.49, positive predictive value (PPV) 0.32, negative predictive value (NPV) 1.00. The PPV for the different grades was 0.00 for Grade 1, 0.56 for Grade 2, 1.00 for Grade 3; the NPV of Grade 0 was 1.00. A significant correlation was found between the CE grading and the number of PAVM, and complications (P < 0.0001). CONCLUSION: CE is an extremely sensitive procedure for the detection of PAVMs with substantial clinical impact.

Heart rate and blood pressure response to upright tilt in young patients with unexplained syncope.

Syncope in apparently normal patients has been attributed to an inhibitory reflex originating in cardiac sensory receptors. The reflex may be elicited by upright tilt with or without isoproterenol infusion. In this study, an upright 90 degree tilt protocol was evaluated in 20 young patients aged 7 to 22 years with syncope but with normal cardiac and neurologic evaluations. The electrocardiogram and blood pressure were noninvasively recorded at 1 min intervals while the patient was supine (5 to 10 min) and during tilt (15 min) in the baseline state. The protocol was repeated during isoproterenol infusion at increasing doses until symptoms of syncope or near syncope were provoked or the maximal isoproterenol dose was achieved (0.07 to 0.1 microgram/kg per min). Mean heart rate, mean blood pressure and RR interval variability, expressed as the standard deviation and the mean of the absolute difference between consecutive RR intervals, were assessed. Symptoms were elicited during tilt in 16 of the 20 patients (in 4 at baseline and in 12 with isoproterenol infusion); no symptoms were induced in 4 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Tailoring the hybrid palliation for hypoplastic left heart syndrome: A simulation study using a lumped parameter model.

The results of Hybrid procedure (HP) for the hypoplastic left heart syndrome (HLHS) depend on several variables: pulmonary artery banding tightness (PAB), atrial septal defect size (ASD) and patent ductus arteriosus stent size (PDA). A HP complication could be the aortic coarctaction (CoAo). The reverse Blalock-Taussig shunt (RevBT) placement was proposed to avoid CoAo effects. This work aims at developing a lumped parameter model (LPM) to investigate the effects of the different variables on HP haemodynamics. A preliminary verification was performed collecting measurements on a newborn HLHS patient to calculate LPM input parameters to reproduce patient's baseline. Results suggest that haemodynamics is affected by ASD (ASD: 0.15-0.55 cm, pulmonary to systemic flow ratio Qp/Qs: 0.73-1, cardiac output (CO): 1-1.5 l/min and ventricular stroke work SW: 336-577 ml mmHg) and by the PAB diameter (PAB: 0.07-0.2 cm, Qp/Qs: 0.46-2.1, CO: 1.3-1.6 l/min and SW: 591-535 ml mmHg). Haemodynamics was neither affected by RevBT diameter nor by PDA diameter higher than 0.2 cm. RevBT implantation does not change the HP haemodynamics, but it can make the CoAo effect negligible. LPM could be useful to support clinical decision in complex physiopathology and to calibrate and personalise the parameters that play a role on flow distribution.

Use of amiodarone for short-term and adjuvant therapy in young patients.

Limited data are available defining the safety of amiodarone for short-term use or as part of combination antiarrhythmic therapy in pediatric patients. Results of amiodarone in 47 young patients for an average treatment duration of 12 months were examined. There were 21 male and 26 female patients (age range of 23 weeks gestation to 29 years). Patients were divided into 4 groups: group 1--electrocardiographic documented ventricular tachycardia (n = 7); group 2--syncope of unknown cause (n = 16); group 3--primary atrial tachycardia (n = 11); and group 4--supraventricular tachycardia (n = 13). Amiodarone was clinically useful in 32 (68%) patients. Amiodarone was considered effective as a sole antiarrhythmic agent in 21 (45%) patients. Treatment was ineffective but was continued in 11 (23%) patients; in 10 of these 11 patients amiodarone was adjuvant to other antiarrhythmic drugs. Amiodarone was considered ineffective and was withdrawn in 15 (32%) patients. No patient required cardiac pacemaker implant during therapy. Torsades de pointes and cardiac arrest occurred in 1 patient each after 9 and 14 days of therapy, respectively. Two patients underwent successful cardiac transplant after 2 and 14 months of amiodarone administration, respectively. Amiodarone was used as short-term treatment (less than 18 months) in 7 infants (age less than 18 months), and after cessation of treatment there was no recurrence of tachycardia for 4 to 24 months. Results of this study confirm reports of successful amiodarone use in pediatric patients with a variety of rhythm disturbances.(ABSTRACT TRUNCATED AT 250 WORDS)

Mechanism of acquired right ventricular outflow tract obstruction in patients with ventricular septal defect: an angiocardiographic study.

Right ventricular outflow tract obstruction can complicate the natural history of patients with isolated ventricular septal defect. Although numerous pathologic mechanisms may be responsible for this phenomenon, it is less clear what the frequency is of each of these mechanisms in any cohort of patients studied in a single institution. We identified 20 patients with isolated ventricular septal defect from 1970 to 1981 who acquired a right ventricular outflow tract gradient of 25 mm Hg or more. They ranged in age at the initial catheterization investigation from 4 days to 23 months and at the subsequent study from 13 months to 8 years. Angiographically, 2 mechanisms were implicated in the 20 patients. Hypertrophy of right ventricular anomalous muscle bundles was identified in 19 of the 20 while hypertrophy of a malaligned infundibular septum (that is, classic tetralogy type) was identified in only 1 patient. These data indicate that acquired right ventricular outflow tract obstruction is rarely of the classic tetralogy type.

Role of transesophageal pacing in evaluation of palpitations in children and adolescents.

Transesophageal atrial pacing was used to evaluate the cause of palpitations in 28 patients ages 3 to 18 years (mean 11). Palpitations were defined as the sustained (seconds to minutes) sensation of rapid heart beating. Each patient had had greater than 2 episodes of palpitations. No patient had other evidence of heart disease. Standard electrocardiogram was normal (23 of 28 patients), demonstrated ventricular preexcitation (3 of 28 patients) or demonstrated short PR interval (2 of 28 patients). In selected patients, ambulatory monitoring (11 patients) or exercise testing (3 patients) was performed but failed to demonstrate a cause of palpitations. In an effort to initiate tachycardia, a similar transesophageal atrial pacing protocol was performed in each patient. The protocol consisted of: (1) single extrastimuli at progressively closer intervals during sinus rhythm and after an 8-beat pacing train at greater than or equal to 1 cycle lengths and (2) incremental atrial pacing to the point of second-degree atrioventricular block. If this pacing regimen failed to initiate tachycardia, it was repeated during isoproterenol infusion (0.02, 0.05 and 0.1 micrograms/kg/min) and then following intravenous atropine (0.04 mg/kg) administration. During the study, tachycardia was initiated in 20 of 28 patients (71%) (14 of 15 patients greater than 10 years, 6 of 13 patients less than or equal to 10 years; p less than 0.01, Fisher's exact test). Electrophysiologic characteristics of induced tachycardia suggested reentry within the atrioventricular node (8 of 20 patients) or orthodromic reciprocating tachycardia (12 of 20 patients). In 3 of 12 patients with orthodromic reciprocating tachycardia, a transition to atrial fibrillation was observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Randomized comparison of atenolol and fludrocortisone acetate in the treatment of pediatric neurally mediated syncope.

Overall, these results indicate that oral treatment of neurally mediated syncope is safe and efficacious. Further randomized trials in children will be required to determine the significance of a placebo effect, as well as potential differences in results related to the mechanism of syncope.

Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients.

Pulmonary vein wedge angiography was applied to 25 patients ranging in age from 4 months to 16 years. The indications for this technique include (1) determination of the presence or absence of central (intrapericardial or mediastinal) pulmonary arteries in patients with pulmonary atresia where standard anterograde injections of contrast medium do not demonstrate these, (2) determination of the patency and caliber of a nonvisualized pulmonary artery when there is distal "occlusion" by a systemic-to-pulmonary artery anastomosis, (3) determination of the patency and caliber of a nonvisualized pulmonary artery when previous pulmonary arterial banding has led to acquired pulmonary artery atresia, and (4) determination of the presence of a mediastinal pulmonary artery in patients with so-called isolated congenital unilateral absence of a pulmonary artery. Surgical correlates to the interpretation of pulmonary vein wedge angiograms are provided in 18 patients. The present data suggest that pulmonary vein wedge angiography is a safe technique that provides considerable information about (1) the caliber of the parenchymal pulmonary arteries, (2) their sizes at the hilum of the lung, and (3) the presence or absence of a mediastinal confluence of pulmonary arteries. This technique may also complement those anatomic findings derived from anterograde injection of contrast material in patients with pulmonary atresia of a congenital or acquired nature.

Palliative right ventricular outflow tract construction for patients with pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries.

From December, 1977, through June, 1981, 15 patients with pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries underwent a palliative right ventricular outflow tract construction. In seven (Group A) this was the first surgical procedure, whereas in the remaining eight (Group B) previous systemic-pulmonary artery anastomoses had been performed. A high morbidity of the pulmonary arteries was present in Group B, resulting in part from the previous shunting procedures. The left pulmonary artery was severely hypoplastic in five patients; the right was hypoplastic in seven of the eight patients and was severely stenotic or atretic in three. All the patients in Group A survived the palliative right ventricular outflow tract reconstruction, and only one in Group B died. Almost all patients had an increase in the aortic PO2 and oxygen saturations after palliative right ventricular outflow tract construction, with a concomitant fall in hemoglobin concentration. Three of the five patients in Group A who underwent postoperative angiography were found to have proximal left pulmonary artery stenosis, and three had lesser involvement of the proximal right pulmonary artery as well. Three of the six patients in Group B who were studied postoperatively also had severe stenosis or proximal atresia of the right pulmonary artery, and three patients had stenosis and hypoplasia of the left pulmonary artery as well, two severe. Thus in no patient in this series were normal pulmonary arteries achieved by palliative right ventricular outflow tract construction will provide the potential for ultimate complete repair. The rather constant finding of severe proximal left pulmonary artery stenosis and hypoplasia in Group A is disappointing.

Recurrent unilateral bacterial pneumonias and interstitial fibrosis associated with pulmonary vein atresia: successful treatment with endovascular stent implantation.

A variety of pulmonary vascular disorders, such as hemangiomatosis, telangectasia, and veno-occlusive disease, may be involved in the pathogenesis of interstitial lung diseases. We describe the case of a girl with recurrent bacterial pneumonia and progressive interstitial fibrosis affecting the right lung. Morphologic evaluation of the lung biopsy showed structural changes of the vessel walls suggesting pulmonary hypertension. The echocardiogram showed the presence of centripetal blood flow in the right pulmonary artery from the periphery of the lung to the heart. A selective right angiography demonstrated the presence of pulmonary venous obstruction at the veno-atrial junction, successfully treated by endovascular stent implantation during cardiac catheterization.

Myocardial ischaemia un-masked by transesophageal atrial pacing combined with two-dimensional transthoracic echocardiography in a pediatric patient: a case report.

We describe a case of a child operated on for an anomalous origin of the left coronary artery from the pulmonary artery and proximal hypoplasia of the anomalous coronary, in whom residual inducible myocardial ischaemia was detected by means of transesophageal atrial pacing combined with transthoracic echocardiography.

Interruption of the aortic arch, ventricular septal defect, aortic atresia and aortopulmonary fistulous communication.

Interruption of the aortic arch in association with aortic atresia is a rare condition. We report the second case in literature in which survival was provided by an aortopulmonary fistulous communication.

Ventricular assist devices as a bridge to heart transplantation or as destination therapy in pediatric patients.

PURPOSE: Despite the remarkable advances with the use of ventricular assist devices (VAD) in adults, pneumatic pulsatile support in children is still limited. We report on our experience in the pediatric population. METHODS: Retrospective review of 27 consecutive children offered mechanical support with Berlin Heart as a bridge to heart transplant, and Jarvik 2000 as a destination therapy from February 2002 to October 2011. RESULTS: The median patient age was 4.8 years (range = 75 days to 20.5 years). The median patient weight was 18.6 kg (range = 2.9-63 kg). We divided the patients in two groups, including in group I patients assisted for bridging to heart transplantation and in group II patients with Duchenne's dystrophy assisted as destination therapy. In the group I, 11 patients required biventricular mechanical support (BVAD), but in all other cases, a single left VAD proved sufficient (56%). The median duration of VAD support was 48 days (1 to 192 days). The median pre-VAD pulmonary vascular resistance index (Rpi) was 5.7 WU/m(2) (3.5 to 14.4 WU/m(2)). Twelve patients (48%) were successfully bridged to heart transplantation after a median duration of mechanical support of 63 days (range = 2-168 days). Ten deaths occurred (40%), three for neurological complications, two for sepsis, two for multiorgan failure, and three other for device malfunctioning. Since 2007, the survival rate of our patients has increased from 33% to 75%, and the need for BVAD has decreased from 89% to 23%. In the group II, two patients with mean age of 15.3 years were assisted with Jarvik 2000, and both of them are alive in a follow-up of 10.4 months. In two patients with Rpi > 10 WU/m(2), unresponsive to pulmonary vasodilatator therapy, Rpi dropped to 2.2 and 2 WU/m(2) after 40 and 23 days of BVAD support, respectively. Six patients (32%) required at least one pump change. Of 12 patients undergoing heart transplantation, five developed an extremely elevated (>60%) panel-reactive antibody by enzyme-linked immunosorbent assay, confirmed by Luminex. All of them experienced at least one acute episode of rejection in the first month after heart transplant, needing plasmapheresis. The survival rate after heart transplantation was 100% with a median follow-up of 34.4 months (45 days to 8.7 years). CONCLUSIONS: Mechanical support in children with end-stage heart failure is an effective strategy as a bridge to heart transplantation with a reasonable morbidity and mortality. BVAD support may offer an additional means to reverse extremely elevated pulmonary vascular resistance. The total implantable system opens a future scenarios for patients not eligible for heart transplantation.

A statistical model for quantification and prediction of cardiac remodelling: application to tetralogy of Fallot.

Cardiac remodelling plays a crucial role in heart diseases. Analyzing how the heart grows and remodels over time can provide precious insights into pathological mechanisms, eventually resulting in quantitative metrics for disease evaluation and therapy planning. This study aims to quantify the regional impacts of valve regurgitation and heart growth upon the end-diastolic right ventricle (RV) in patients with tetralogy of Fallot, a severe congenital heart defect. The ultimate goal is to determine, among clinical variables, predictors for the RV shape from which a statistical model that predicts RV remodelling is built. Our approach relies on a forward model based on currents and a diffeomorphic surface registration algorithm to estimate an unbiased template. Local effects of RV regurgitation upon the RV shape were assessed with Principal Component Analysis (PCA) and cross-sectional multivariate design. A generative 3-D model of RV growth was then estimated using partial least squares (PLS) and canonical correlation analysis (CCA). Applied on a retrospective population of 49 patients, cross-effects between growth and pathology could be identified. Qualitatively, the statistical findings were found realistic by cardiologists. 10-fold cross-validation demonstrated a promising generalization and stability of the growth model. Compared to PCA regression, PLS was more compact, more precise and provided better predictions.

Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum.

OBJECTIVE: To review the outcome of patients with pulmonary atresia with intact ventricular septum after interventional perforation of the pulmonary valve, to assess the capability of this procedure to avoid neonatal or late intervention and to obtain a long-term biventricular repair. DESIGN: Retrospective interventional study and clinical follow-up study. SETTING: Tertiary referral centre. PATIENT POPULATION: Between November 1994 and December 2007, 40 neonates underwent radiofrequency perforation. Median age at pulmonary valvotomy was 28 hours (range 1-147 hours) and median weight was 2925 g (range 1900-4400 g). MAIN OUTCOME MEASURES: Procedural success and complication rates; early-term and long-term follow-up results. RESULTS: The procedure was successful in 39 patients but 16 of them needed neonatal surgery. The overall mortality was 7.5%. At a median follow-up of 82 months, four patients underwent a bidirectional Glenn procedure, whereas all the other patients achieved a biventricular circulation without any further intervention in 19 of them. Patients who died or needed additional intervention with or without biventricular circulation failure had a higher incidence of bipartite right ventricular (65% vs 15.8% of those not needing additional intervention; p = 0.004) and a lower median tricuspid Z value (-2 (range -3.5 to 1) vs -0.5 (range -2 to 1); p = 0.004)). CONCLUSIONS: The results confirm that percutaneous interventional perforation is an effective first-stage procedure in patients with pulmonary atresia with intact ventricular septum. The right heart appeared to be adequate to maintain a long-term biventricular circulation in the large majority of cases.

Exercise capacity in apparently healthy survivors of cancer.

AIMS: To evaluate cardiopulmonary exercise tolerance in a large cohort of apparently healthy paediatric cancer survivors in order to determine their participation in sporting activities. METHODS: A total of 84 young (<21 years) asymptomatic childhood cancer survivors, who had been exposed to anthracyclines (mean dose 212 mg/m2) and/or chest irradiation (median dose 2000 cGy), with normal left ventricular systolic function at rest (fractional shortening >29%), and 79 healthy controls were studied. Exercise testing was performed on a treadmill ergometer. Gas exchange analysis and derived variables were measured on a breath-by-breath basis. Pulmonary functional evaluation was performed before exercise. Echocardiographic evaluation at rest was performed within one month before the exercise test. RESULTS: There were no differences in exercise responses between patients and controls. In boys <13 years, mean VO2 max was slightly but significantly lower than in controls. This finding was thought to be a result of decreased physical fitness as all the other exercise parameters were similar to those in the controls. CONCLUSIONS: Results show that apparently healthy survivors of paediatric cancer can take part in dynamic sporting activities if they exhibit a normal response to cardiopulmonary exercise testing, while those that exhibit a reduced VO2 max should be re-evaluated after an aerobic training programme, and should undergo tailored dynamic physical activity if the VO2 max does not normalise.