Pediatric Cardiology


Pediatric Cardiology

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Zambelli V.,University of Milan Bicocca | Botta G.,Presidio | Moltrasio F.,University of Milan Bicocca | Cattoretti G.,University of Milan Bicocca | Fesslova V.,Pediatric Cardiology
Ultrasound in Obstetrics and Gynecology | Year: 2014

Objective To assess the feasibility and utility of contrast-enhanced microcomputed tomography (micro-CT) for identifying structural anomalies in ex-vivo first- and second-trimester human fetuses and isolated fetal hearts.Methods Radiopaque iodine staining and micro-CT scanning protocols were first developed in rodent studies and then used to examine routinely fixed whole human fetuses (n = 7, weight 0.1-90 g, gestational age, 7-17 weeks) and isolated fetal hearts (n = 14, weight 0.1-5.2 g, gestational age, 11-22 weeks). Samples were scanned using an isotropic resolution of 18 (and, if necessary, 9 or 35) μm and findings were interpreted jointly by four fetal pathologists, a fetal cardiologist and a radiologist. Samples with gestational ages ≥ 13 weeks also underwent conventional autopsy or dissection.Results Micro-CT identified all anatomical structures and abnormalities documented by the macroscopic examination. In all seven cases involving fetuses ≤ 13 weeks (four fetuses, three isolated hearts), micro-CT excluded the presence of structural anomalies. In the remaining 14 cases, it provided all the information obtained with invasive autopsy or dissection and in seven of the 14 (two fetuses, five isolated hearts) it furnished additional diagnostic details.Conclusions This pilot study confirms the feasibility of postmortem contrast-enhanced micro-CT assessment of structural anomalies in whole small fetuses and fetal hearts. Further study is needed to confirm our findings, particularly in whole fetuses, and to define the extent to which this virtual examination might be used instead of conventional invasive autopsy. Copyright © 2014ISUOG. Published by John Wiley & Sons Ltd.

Although historically the intra-aortic balloon pump has been the only mechanical circulatory support device available to clinicians, a number of new devices have become commercially available and have entered clinical practice. These include axial flow pumps, such as Impella®; left atrial to femoral artery bypass pumps, specifically the TandemHeart; and new devices for institution of extracorporeal membrane oxygenation. These devices differ significantly in their hemodynamic effects, insertion, monitoring, and clinical applicability. This document reviews the physiologic impact on the circulation of these devices and their use in specific clinical situations. These situations include patients undergoing high-risk percutaneous coronary intervention, those presenting with cardiogenic shock, and acute decompensated heart failure. Specialized uses for right-sided support and in pediatric populations are discussed and the clinical utility of mechanical circulatory support devices is reviewed, as are the American College of Cardiology/American Heart Association clinical practice guidelines. © 2015 The Society for Cardiovascular Angiography and Interventions, The American College of Cardiology Foundation, The Heart Failure Society of America, and The Society for Thoracic Surgery.

Fairfax S.T.,Biomedical science | Holwerda S.W.,Medical Pharmacology and Physiology | Credeur D.P.,Medical Pharmacology and Physiology | Zuidema M.Y.,Internal Medicine | And 7 more authors.
Journal of Physiology | Year: 2013

Sympathetic vascular transduction is commonly understood to act as a basic relay mechanism, but under basal conditions, competing dilatory signals may interact with and alter the ability of sympathetic activity to decrease vascular conductance. Thus, we determined the extent to which spontaneous bursts of muscle sympathetic nerve activity (MSNA) mediate decreases in forearm vascular conductance (FVC) and the contribution of local α-adrenergic receptor-mediated pathways to the observed FVC responses. In 19 young men, MSNA (microneurography), arterial blood pressure and brachial artery blood flow (duplex Doppler ultrasound) were continuously measured during supine rest. These measures were also recorded in seven men during intra-arterial infusions of normal saline, phentolamine (PHEN) and PHEN with angiotensin II (PHEN+ANG). The latter was used to control for increases in resting blood flow with α-adrenergic blockade. Spike-triggered averaging was used to characterize beat-by-beat changes in FVC for 15 cardiac cycles following each MSNA burst and a peak response was calculated. Following MSNA bursts, FVC initially increased by +3.3 ± 0.3% (P= 0.016) and then robustly decreased to a nadir of -5.8 ± 1.6% (P < 0.001). The magnitude of vasoconstriction appeared graded with the number of consecutive MSNA bursts; while individual burst size only had a mild influence. Neither PHEN nor PHEN+ANG infusions affected the initial rise in FVC, but both infusions significantly attenuated the subsequent decrease in FVC (-2.1 ± 0.7% and -0.7 ± 0.8%, respectively; P < 0.001 vs. normal saline). These findings indicate that spontaneous MSNA bursts evoke robust beat-by-beat decreases in FVC that are exclusively mediated via α-adrenergic receptors. © 2013 The Physiological Society.

Kutty S.,University of Nebraska at Omaha | Kuehne T.,Pediatric Cardiology | Gribben P.,University of Nebraska at Omaha | Reed E.,University of Nebraska at Omaha | And 4 more authors.
Circulation: Cardiovascular Imaging | Year: 2012

Background-Cardiac magnetic resonance (CMR) imaging is an important clinical tool for serial follow-up of patients with congenital heart disease, but normative data for great vessel dimensions in pediatric subjects are scarce. We investigated the ascending aortic (AO) and main pulmonary artery dimensions in normal children and young adults in comparison with a cohort of patients with repaired tetralogy of Fallot (TOF). Methods and Results-Subjects were prospectively enrolled for cardiac magnetic resonance after a standardized protocol in 14 participating centers of the German Competence Network for Congenital Heart Defects. All studies were performed in 1.5-T scanners and used single-slice multiphase acquisitions steady-state free precession and velocity-encoded cine. AO and main pulmonary artery areas were measured. The cohort consisted of 483 subjects: 105 normal controls (55 men; 50 women; and median age, 14 years) and 378 patients with repaired TOF (210 men; 168 women; and median age, 16 years). Among TOF, 35 (9%) had pulmonary atresia, 98 (26%) had a palliative procedure before repair, the mean age at repair was 2.9 years, and 82 (23%) used a transannular patch repair. Great vessel areas correlated well with body surface area and age in controls and reference Z-score values were derived. Z scores for ascending AO areas were larger in TOF compared with controls (mean Z score =1.95, P=0.001). In TOF, pulmonary atresia (P=0.003), male sex (P=0.01) and previous palliations (P=0.046) were associated with larger AO areas. Main pulmonary artery area Z scores in surgically modified TOF were smaller on an average than controls (mean Z score =?0.293 P=0.001) but not small to the same extent as the AO was large. Conclusions-This study provides cardiac magnetic resonance reference Z scores for great vessel areas in normal children and adolescents in comparison with a large contemporary cohort of repaired TOF. Male sex, pulmonary atresia, and previous palliations emerged as predictors for larger AO dimensions in TOF. Clinical Trial Registration-URL: Unique identifier: NCT00266188. © 2012 American Heart Association, Inc.

Butera G.,Pediatric Cardiology and GUCH Unit | Marini D.,Pediatric Cardiology | Carminati M.,Pediatric Cardiology and GUCH Unit
Journal of Invasive Cardiology | Year: 2010

We report on three cases of primary endovascular bare stenting for native aortic coarctation complicated by aortic aneurysm or in-stent stenosis because of neointimal proliferation that were successfully treated by covered stent. These cases show how the deployment of a covered stent may be a safe and definitive therapeutical option in the management of these complications.

Butera G.,Pediatric Cardiology | Milanesi O.,University of Padua | Spadoni I.,Pediatric Cardiology | Piazza L.,Pediatric Cardiology | And 6 more authors.
Catheterization and Cardiovascular Interventions | Year: 2013

Background and aims Percutaneous implantation of pulmonary valve has been recently introduced in the clinical practice. Our aim was to analyze data of patients treated in Italy by using the Melody Medtronic valve. Methods Prospective, observational, multi-centric survey by means of a web-based database registry of the Italian Society of Pediatric Cardiology (SICP). Results Between October 2007 and October 2010, 63 patients were included in the registry (median age: 24 years; range 11-65 years). Forty subjects were in NYHA class I-II while 23 were in NYHA class III-IV. Patients included had a history of a median three previous surgeries (range 1-5) and a median of one previous cardiac catheterization (range 0-4). A cono-truncal disease was present in 39 patients, previous Ross operation in 9, and other diagnosis in 15. Indication to valve implantation was pure stenosis in 21 patients, pure regurgitation in 12, association of stenosis and regurgitation in 30. Implantation was performed in 61 subjects (97%). Pre-stenting was performed in 85% of cases. Median procedure time was 170 minutes (range 85-360). No significant regurgitation was recorded after procedure while the trans-pulmonary gradient reduced significantly. Early major complications occurred in seven subjects (11%). One death occurred in the early post-operative period in a severely ill subject. At a median follow-up of 30 months (range 12-48 months), three patients died due to underlying disease. Major complications occurred in six patients during follow-up (external electric cardioversion: one patient; herpes virus encephalitis: two patients; Melody valve endocarditis needing surgical explant: two patients; major fractures of the stent and need second Melody valve implantation: two patients). Freedom from valve failure at latest follow-up was 81.4% ± 9%. Conclusion Early results of the SICP registry on transcatheter Melody pulmonary valve implantation show that the procedure is safe and successful. Major concerns are related to the occurrence of stent fracture and bacterial endocarditis. Longer follow-up and larger series are needed. Copyright © 2012 Wiley Periodicals, Inc.

Rosti L.,Pediatric Cardiology
La Pediatria medica e chirurgica : Medical and surgical pediatrics | Year: 2011

Calprotectin is a protein released into stools, used as a marker of inflammation in inflammatory bowel diseases. We tested the hypothesis that cow's milk protein in formula milk may increase the intestinal release of calprotectin, as a consequence of a subclinical inflammatory reaction. At 12 weeks of age, we measured fecal calprotectin by an immunoenzyme assay (Calprest, Eurospital, Trieste, Italy), in 38 exclusively breastfed and in 32 exclusively formula-fed infants. Fecal calprotectin levels were not different in the two groups (p = 0.09), although a trend to higher values in infants with colic, or with family history of allergies was noted. This suggest that, in general, formula milk does not promote activation of an intestinal inflammatory reaction, compared to human milk, although a subclinical activation of the inflammatory response in infants at risk for allergic diseases may be present.

Sciacca P.,Pediatric Cardiology | Giacchi V.,AOU Policlinico Vittorio Emanuele | Mattia C.,NICU | Greco F.,AOU Policlinico Vittorio Emanuele | And 3 more authors.
BMC Cardiovascular Disorders | Year: 2014

Background: Rhabdomyomas are the most common type of cardiac tumors in children. Anatomically, they can be considered as hamartomas. They are usually randomly diagnosed antenatally or postnatally sometimes presenting in the neonatal period with haemodynamic compromise or severe arrhythmias although most neonatal cases remain asymptomatic. Typically rhabdomyomas are multiple lesions and usually regress spontaneously but are often associated with tuberous sclerosis complex (TSC), an autosomal dominant multisystem disorder caused by mutations in either of the two genes, TSC1 or TSC2. Diagnosis of tuberous sclerosis is usually made on clinical grounds and eventually confirmed by a genetic test by searching for TSC genes mutations.Methods: We report our experience on 33 cases affected with rhabdomyomas and diagnosed from January 1989 to December 2012, focusing on the cardiac outcome and on association with the signs of tuberous sclerosis complex. We performed echocardiography using initially a Philips Sonos 2500 with a 7,5/5 probe and in the last 4 years a Philips IE33 with a S12-4 probe. We investigated the family history, brain, skin, kidney and retinal lesions, development of seizures, and neuropsychiatric disorders.Results: At diagnosis we detected 205 masses, mostly localized in interventricular septum, right ventricle and left ventricle. Only in 4 babies (12%) the presence of a mass caused a significant obstruction. A baby, with an enormous septal rhabdomyoma associated to multiple rhabdomyomas in both right and left ventricular walls died just after birth due to severe heart failure. During follow-up we observed a reduction of rhabdomyomas in terms of both number and size in all 32 surviving patients except in one child. Eight patients (24,2%) had an arrhythmia and in 2 of these cases rhabdomyomas led to Wolf-Parkinson-White Syndrome. For all patients the arrhythmia spontaneously totally disappeared or was reduced gradually. With regarding to association with tuberous sclerosis, we diagnosed tuberous sclerosis clinically in 31 babies (93,9%).Conclusion: Rhabdobyomas are tumors with favorable prognosis because they frequently do not cause symptoms and they often regress in numbers and size. Nevertheless, due to frequent association with tuberous sclerosis complex and the resulting neurological impairment, the prognosis can result unfavorable. © 2014 Sciacca et al.; licensee BioMed Central Ltd.

Tristani-Firouzi M.,Pediatric Cardiology | Tristani-Firouzi M.,University of Utah
Journal of Electrocardiology | Year: 2015

The incidence of long QT syndrome (LQTS) is generally believed to be approximately 1:2500 individuals. Given the relatively common nature of this disorder, the observation that sudden cardiac death is often a presenting symptom and the highly treatable nature of the disease, LQTS meets common criteria for the design of a universal screening program. In spite of multiple lines of evidence (international experience, cost-benefit simulations) suggesting that universal screening for LQTS is feasible and effective, there remains considerable debate and controversy surrounding the implementation of universal LQTS screening in the United States. Here, we describe some of the challenges and potential novel solutions to the practical implementation of LQTS screening. © 2015 Elsevier Inc. All rights reserved.

Kefer J.,Catholic University of Louvain | Sluysmans T.,Pediatric Cardiology | Vanoverschelde J.-L.,Catholic University of Louvain
Catheterization and Cardiovascular Interventions | Year: 2014

Objectives We describe the first report of a transcatheter Sapien implantation in a native tricuspid valve after multiple failed surgical repairs with a lack of prosthetic material and radiographic landmarks. Methods and Results A 47-year old female underwent multiple valve repairs and replacements including three tricuspid valve repairs without surgical ring or bioprosthesis implantation. She developed signs of right heart failure associated with a mixed tricuspid disease combining a severe stenosis and regurgitation. After surgical turn down, a revalvulation using a transcatheter approach was attempted the challenges in this case were the absence of a stiff region to anchor the percutaneous valve, the lack of radiographic landmarks and the difficulties of precise annulus measurements the applied strategy was -under general anesthesia and extracorporeal membrane oxygenation-: balloon sizing, prestenting of the tricuspid annulus using covered stents followed by Sapien valve implantation through the femoral vein under fluoroscopy and transoesophageal echocardiography (TEE) the procedure was successfull, solving the tricuspid leak and stenosis (peak gradient from 22 to 3 mm Hg) using two Sapien for a perfect positioning. It was complicated by pulmonary bleeding due to a distal wire exit, treated successfully by coil embolization the clinical and echocardiographic outcome was good up to 5 months. Conclusions Transcatheter Sapien valve implantation in a native tricuspid valve after failed multiple surgical repairs is feasible by the femoral vein. Technical challenges due to the lack of rigid landing zone and fluoroscopic markers were solved by prestenting and valve implantation under bi-plane fluoroscopic and TEE guidance. © 2013 Wiley Periodicals, Inc. Copyright © 2013 Wiley Periodicals, Inc.

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