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1.
Front Pediatr ; 9: 707760, 2021.
Article in English | MEDLINE | ID: mdl-34291021

ABSTRACT

Kingella kingae is a gram-negative coccobacillus belonging to the HACEK group (Haemophilus species, Aggregatibacter actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, and Kingella species) and is a common oropharyngeal colonizer of healthy young children. Osteoarticular infection is the most commonly reported invasive Kingella kingae infection in children, usually presenting a mild clinical picture. However, it can also cause severe invasive infections, especially infective endocarditis, with a high complication rate. We report the case of a 6-year-old girl, with no past medical history, who presented with fulminant infective endocarditis due to Kingella kingae. She received emergency venoarterial extracorporeal membrane oxygenation support, rapidly underwent cardiac surgery, and was then treated using ceftriaxone for 4 weeks as recommended by the American Heart Association. The patient's postoperative course was marked by a cerebral ischemic stroke consistent with septic embolism. She also presented with a para-aortic pseudoaneurysm that required a secondary surgical procedure, with a good postoperative result. This report illustrates a case of fulminant infective endocarditis due to Kingella kingae and responsible for two major complications. We also describe the preventive valve surgery performed to ensure the preservation of valve function and its capacity for growth.

2.
J Hepatol ; 74(3): 742-747, 2021 03.
Article in English | MEDLINE | ID: mdl-33276028

ABSTRACT

Portopulmonary hypertension is a rare but serious complication of portal hypertension or portosystemic shunting. Portopulmonary hypertension is an indication for liver transplantation or shunt closure. However, liver transplantation is contraindicated in patients with severe pulmonary arterial hypertension. Reported mortality rates are high in children with portopulmonary hypertension and there are scarce recommendations on its management. Our aim was to report on our real-world experience of managing portopulmonary hypertension in a specialised centre. We describe a series of 6 children with portopulmonary hypertension. Their median age at diagnosis was 13 years (range 10-15). The underlying liver conditions were cirrhosis of unknown origin (1), congenital portocaval shunts (3), biliary atresia (1), and portal vein cavernoma with surgical mesenterico-caval shunt (1). Median mean pulmonary arterial pressure was 47 mmHg (range 32-70), and median pulmonary vascular resistance was 6.6 Wood units (range 4.3-15.4). All patients except one were treated with a combination of pulmonary arterial hypertension-specific therapy (phosphodiesterase type 5 inhibitors and/or endothelin receptor antagonists and/or prostacyclin analogues). Three patients then benefited from shunt closure and the others underwent liver transplantation. Five patients showed improvement or stabilisation of pulmonary arterial hypertension with no deaths after a mean follow-up of 39 months. Based on our limited experience, early and aggressive treatment with a combination of pulmonary arterial hypertension-specific therapy significantly improves patients' haemodynamic profile and enables the performance of liver transplantation and shunt closure with satisfactory outcomes.


Subject(s)
Antihypertensive Agents/therapeutic use , Endothelin Receptor Antagonists/therapeutic use , Epoprostenol/therapeutic use , Hypertension, Portal/complications , Hypertension, Portal/drug therapy , Liver Cirrhosis/complications , Liver Transplantation/methods , Phosphodiesterase 5 Inhibitors/therapeutic use , Portasystemic Shunt, Surgical/methods , Pulmonary Arterial Hypertension/complications , Pulmonary Arterial Hypertension/drug therapy , Adolescent , Child , Female , Follow-Up Studies , Humans , Hypertension, Portal/surgery , Male , Portal Vein/physiopathology , Pulmonary Arterial Hypertension/surgery , Treatment Outcome
3.
Chest ; 158(6): e295-e298, 2020 12.
Article in English | MEDLINE | ID: mdl-33280772

ABSTRACT

CASE PRESENTATION: A 13-year-old male was referred after incidental finding of cardiomegaly on chest radiograph and signs of pulmonary hypertension on subsequent cardiology consult. He was diagnosed with idiopathic pulmonary hypertension, and came to our center for a second opinion. He was born from consanguineous parents. He reported to be asymptomatic in his daily life. He was not on medications. Family history was not contributive.


Subject(s)
Portal Vein/abnormalities , Pulmonary Arterial Hypertension/etiology , Vascular Malformations/complications , Vena Cava, Inferior/abnormalities , Adolescent , Angiography , Diagnosis, Differential , Humans , Male , Portal Vein/diagnostic imaging , Pulmonary Arterial Hypertension/diagnosis , Tomography, X-Ray Computed , Vascular Malformations/diagnosis , Vena Cava, Inferior/diagnostic imaging
5.
Hum Mol Genet ; 29(4): 618-623, 2020 03 13.
Article in English | MEDLINE | ID: mdl-31903486

ABSTRACT

In a consanguineous Pakistani family with two affected individuals, a homozygous variant Gly399Val in the eighth transmembrane domain of the taurine transporter SLC6A6 was identified resulting in a hypomorph transporting capacity of ~15% compared with normal. Three-dimensional modeling of this variant has indicated that it likely causes displacement of the Tyr138 (TM3) side chain, important for transport of taurine. The affected individuals presented with rapidly progressive childhood retinal degeneration, cardiomyopathy and almost undetectable plasma taurine levels. Oral taurine supplementation of 100 mg/kg/day resulted in maintenance of normal blood taurine levels. Following approval by the ethics committee, a long-term supplementation treatment was introduced. Remarkably, after 24-months, the cardiomyopathy was corrected in both affected siblings, and in the 6-years-old, the retinal degeneration was arrested, and the vision was clinically improved. Similar therapeutic approaches could be employed in Mendelian phenotypes caused by the dysfunction of the hundreds of other molecular transporters.


Subject(s)
Cardiomyopathies/drug therapy , Membrane Glycoproteins/deficiency , Membrane Transport Proteins/deficiency , Retinal Degeneration/drug therapy , Taurine/therapeutic use , Adolescent , Biological Transport , Cardiomyopathies/metabolism , Cardiomyopathies/pathology , Child , Female , Humans , Male , Pedigree , Retinal Degeneration/metabolism , Retinal Degeneration/pathology
6.
Clin Obes ; 9(6): e12335, 2019 Dec.
Article in English | MEDLINE | ID: mdl-31415133

ABSTRACT

Childhood obesity results in premature atherosclerosis and requires early intervention. Compare the effectiveness of 6-month lifestyle interventions (with choice of either individual or group therapy) with standard care on body mass index (BMI) z-score and cardiovascular disease (CVD) risks factors in children with obesity. This 6-month randomized controlled trial with a 6-month follow-up included 74 pre-pubertal children with obesity (7.5-11.9 years) assigned randomly (2:1) to intervention or control. Families in the intervention arm choose between an individually delivered treatment (3 hours paediatrician + 4 hours dietician) or group treatment (35 hours with a multidisciplinary team). Children participated also to a weekly physical activity programme. We measured BMI, BMI z-score; waist circumference (WC); total and abdominal fat; blood pressure; common carotid artery intima-media thickness and incremental elastic modulus (Einc); endothelium-dependent and independent dilation (nitroglycerin-mediated dilation [NTGMD]) of the brachial artery; fasting plasma glucose, insulin, lipids; and high-sensitivity C-reactive protein (hs-CRP). Compared to controls, at 6 months, abdominal fat and hs-CRP were reduced in both interventions. The group intervention was also effective in reducing BMI (-0.55 kg/m2 ; 95% confidence interval -1.16 to 0.06) and BMI z-score (-0.08; -0.15 to 0.00) at 6 months and BMI, BMI z-score, WC, NTGMD, total and abdominal fat at 12 months. Abdominal fat and low-grade inflammation were significantly decreased in both interventions. High-intensity group treatment improved early signs of atherosclerosis in children with obesity. These findings are important for the promotion of cardiometabolic health in this population.


Subject(s)
Cardiovascular Diseases/therapy , Pediatric Obesity/therapy , Blood Glucose/metabolism , Blood Pressure , Body Mass Index , C-Reactive Protein/metabolism , Cardiovascular Diseases/metabolism , Cardiovascular Diseases/physiopathology , Cardiovascular Diseases/psychology , Carotid Intima-Media Thickness , Child , Diet, Healthy , Exercise , Exercise Therapy , Female , Humans , Insulin/blood , Life Style , Male , Pediatric Obesity/metabolism , Pediatric Obesity/physiopathology , Pediatric Obesity/psychology , Risk Factors , Treatment Outcome , Waist Circumference
7.
Front Pediatr ; 7: 262, 2019.
Article in English | MEDLINE | ID: mdl-31334208

ABSTRACT

A 5-year-old girl presented with acute nocturnal episodes of loss of consciousness following abdominal pain and crying. Epilepsy was primarily diagnosed but the course of the disease was suggestive of pulmonary hypertension. An adapted invasive assessment of pulmonary pressure and pharmacological challenge allowed for diagnosing vasoreactive pulmonary arterial hypertension. Initial treatment with sildenafil was not effective. Thus, calcium channel blockers were introduced when positive vasoreactivity was confirmed and permitted to stop the occurrence of the syncope and dramatically improved clinical status. At 2 years follow-up she is well without any complaint and in functional class I. Echocardiography shows a slightly enlarged but not hypertrophied right ventricle with a nearly normalized estimated right ventricular pressure. The last catheterization shows subnormal values of pulmonary arterial pressure (mean pulmonary artery pressure: 24 mmHg) and pulmonary vascular resistance (5, 4 Wood units*m2), normalizing with inhaled Nitric Oxide (mean pulmonary artery pressure of 14 mmHg and pulmonary vascular resistance of 1.5 Wood units*m2). Vasoreactive pulmonary arterial hypertension is a rare entity in children but it should not be misdiagnosed with seizures due to the presence of syncopal episodes. According to current knowledge, this form seems to have a better prognosis than non-reactive pulmonary arterial hypertension and the treatment of choice remains as calcium channel blockers. The management of this case was characterized by successive mishaps and potentially harmful mistakes and underscores the potential risk with pediatric PH evaluation in non-expert centers.

8.
Eur J Clin Invest ; 48(9): e12995, 2018 Sep.
Article in English | MEDLINE | ID: mdl-29992540

ABSTRACT

BACKGROUND: Childhood obesity is associated with premature cardiovascular complications. However, little is known about the effect of a family-based behavioural intervention on the relationship between arterial function, blood pressure and biomarkers in pre-pubertal children with obesity. DESIGN: This was a single centre randomized controlled trial (RCT) including 74 children randomized to a 6-month behavioural intervention to treat obesity. In 48 children (13 controls and 35 interventions), we assessed: serum level of cytokine (CCL2), adiponectin, and neutrophil product (MMP-8), as well as carotid intima-media thickness, flow-mediated dilation (FMD), nitroglycerin-mediated dilation; arterial stiffness (incremental elastic modulus, Einc), pulse wave velocity (PWV), resting and 24-hour blood pressure (BP). RESULTS: At baseline, resting systolic BP was positively associated with MMP-8 levels which was significantly higher in children with hypertension (P = 0.033). Biochemical markers were not related to endothelial function at baseline, but they globally increased after 6 months in the intervention group. The significant increase of CCL2 levels in the intervention group was associated with a decrease in diastolic BP. Furthermore, adiponectin change was positively related to a change in FMD and negatively to change in Einc and PWV. CONCLUSIONS: The usefulness of serum biomarkers for the detection of cardiovascular diseases is not well established in children. In our population, MMP-8 concentration was higher in hypertensive children. Furthermore, behavioural interventions resulted in a paradoxical increase in some biomarkers in children, with potentially beneficial effects detected with CCL2 changes. Caution should be taken when using nonspecific serum biomarkers for the clinical monitoring of children with obesity.


Subject(s)
Adiponectin/blood , Cardiovascular Diseases/blood , Chemokine CCL2/blood , Matrix Metalloproteinase 8/blood , Pediatric Obesity/blood , Behavior Therapy , Biomarkers/blood , Blood Pressure/physiology , Blood Pressure Monitoring, Ambulatory , Cardiovascular Diseases/physiopathology , Carotid Intima-Media Thickness , Child , Elastic Modulus , Endothelium, Vascular/physiopathology , Female , Humans , Hypertension/blood , Hypertension/physiopathology , Male , Nitroglycerin , Pediatric Obesity/therapy , Pulse Wave Analysis , Randomized Controlled Trials as Topic , Risk , Vascular Stiffness/physiology , Vasodilation/physiology , Vasodilator Agents
9.
Interact Cardiovasc Thorac Surg ; 27(4): 581-585, 2018 10 01.
Article in English | MEDLINE | ID: mdl-29912349

ABSTRACT

OBJECTIVES: Ventricular retraining and arterial switch have been described in late-presenting transposition of the great arteries (TGA) in older infants who were unable to undergo neonatal arterial switch operation (ASO) and late survivors of atrial switch with systemic right ventricular dysfunction. There are little data available on patients presenting between these 2 groups. This study aims to review the early and mid-term outcomes of the management of late-presenting TGA with an unprepared left ventricle (LV) by a 2-stage arterial switch. METHODS: The demographic, procedural and outcome data were obtained for all children who underwent LV retraining for late-presenting TGA between 2005 and 2017 at our institution. The primary outcomes were early mortality and extracorporeal membrane oxygenation (ECMO) after arterial switch. RESULTS: Twenty patients were included during the study period, with a median age of 12 months (range 6 weeks-3.3 years). The median time of LV retraining was 48 (range 8-170) days. Indexed LV mass increased from 34 ± 19 g/m2 before LV retraining to 106 ± 85 g/m2 before arterial switch. There was 1 death (5%) after LV retraining. Three patients required ECMO support after arterial switch (15%) despite retraining. During follow-up, there was 1 late death, no late reinterventions or reoperations, and all surviving patients had normal or near-normal LV function at late follow-up. CONCLUSIONS: LV retraining resulted in an increase in LV mass and enabled a 2-stage arterial switch to be carried out with acceptable early and mid-term outcomes. Two-stage arterial switch is a reasonable option for late-presenting TGA. A long-term follow-up is required to assess late LV function after preparation.


Subject(s)
Arterial Switch Operation/methods , Heart Ventricles/physiopathology , Transposition of Great Vessels/surgery , Ventricular Function, Left/physiology , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Retrospective Studies , Time Factors , Transposition of Great Vessels/diagnosis , Transposition of Great Vessels/physiopathology , Treatment Outcome
10.
Res Cardiovasc Med ; 5(3): e32545, 2016 Aug.
Article in English | MEDLINE | ID: mdl-27800456

ABSTRACT

INTRODUCTION: The use of extracorporeal membrane oxygenation (ECMO) is considered a risk factor for, or even a potential contraindication to, lung transplantation. However, only a few pediatric cases have been described thus far. CASE PRESENTATION: A 9-year-old boy with idiopathic pulmonary arterial hypertension developed cardiac arrest after the insertion of a central catheter. ECMO was used as a bridge to lung transplantation. However, after prolonged resuscitation, he developed medullary ischemia and medullary syndrome. After 6 weeks of ECMO and triple combination therapy for pulmonary hypertension, including continuous intravenous prostacyclin, he was weaned off support, and after 2 weeks, bilateral lung transplantation was performed. At 4 years post-transplant, he has minimal problems. The medullary syndrome has also alleviated. He is now back to school and can walk with aids. CONCLUSIONS: Increasing evidence supports the use of ECMO as a bridge to LT, reporting good outcomes. In the modern era of PAH therapy, it is feasible to use prolonged ECMO support as a bridge to lung transplant, with the aim of weaning off this support; however, its use requires more experience and knowledge of long-term outcomes.

11.
Cardiol Young ; 25(2): 295-300, 2015 Feb.
Article in English | MEDLINE | ID: mdl-24382023

ABSTRACT

INTRODUCTION: Patients with absent pulmonary valve syndrome often present early with airway compression from aneurysmal pulmonary arteries. This study reviews our experience in managing absent pulmonary valve syndrome in later presenting children, and techniques used for managing airway compression. METHODS: This study is a retrospective chart review of all patients who underwent repair of absent pulmonary valve syndrome from 2000 to 2012 at our institution. The primary endpoints were post-operative bronchoscopic and clinical evidence of persistent airway compression and need for reinterventions on the pulmonary arteries. RESULTS: A total of 19 patients were included during the study period. The mean age at repair was 4.1±3.0 years (range 10 months-11 years). In all, seven patients had pre-operative bronchoscopic evidence of airway compression, which was managed by pulmonary artery reduction plasty in four patients and Lecompte manoeuvre in three patients. There were no peri-operative deaths. In patients with pulmonary artery plasty, two had no post-operative airway compression, one patient had improved compression, and one patient had unchanged compression. In patients managed with a Lecompte manoeuvre, two patients had no or trivial airway compression and one had improved compression. There were six late reinterventions or reoperations on the pulmonary arteries - two out of four in the pulmonary artery plasty group and one out of three in the Lecompte group. CONCLUSIONS: Most late-presenting patients with absent pulmonary valve syndrome do not have airway compression. Either pulmonary artery reduction plasty or the Lecompte manoeuvre can relieve proximal airway compression, without a significantly different risk of pulmonary artery reintervention between techniques.


Subject(s)
Airway Obstruction/etiology , Aneurysm/surgery , Bronchi , Heart Septal Defects, Ventricular/surgery , Heart Valve Diseases/congenital , Pulmonary Artery/surgery , Pulmonary Valve/abnormalities , Trachea , Aneurysm/complications , Bronchoscopy , Child , Child, Preschool , Cohort Studies , Female , Heart Septal Defects, Ventricular/complications , Heart Valve Diseases/complications , Heart Valve Diseases/surgery , Humans , Infant , Male , Reoperation , Retrospective Studies , Syndrome
12.
Eur J Clin Invest ; 42(3): 303-9, 2012 Mar.
Article in English | MEDLINE | ID: mdl-21880038

ABSTRACT

BACKGROUND: Cardiovascular risk markers are related to micro-angiopathy in children with type 1 diabetes (T1DM), but there is no information about their relationship with blood pressure (BP) and endothelial function. MATERIALS AND METHODS: This was a case-control study including 29 children with T1DM (mean age 10·5 ± 2·7 years, disease duration: 3·8 ± 2·2 years) and 39 healthy controls (mean age: 9·8 ± 2·7 years). We assessed 24-h ambulatory BP, vascular function and serum level of lipids, vascular cell adhesion molecule-1 (VCAM-1; ICAM) and selectins (E-selectin; P-selectin). RESULTS: The subject groups had similar physical characteristics and lipids level, except body mass index (BMI) which was higher in T1DM than in healthy children (18·6 ± 2·6 vs. 16·7 ± 2·5 kg/m(2), P = 0·003). Children with T1DM had increased 24 h diastolic BP z-score (0·62 ± 0·9 vs. -0·65 ± 0·8, P < 0·001), even after adjustment for BMI, as well as higher VCAM-1 concentration (492 ± 346 vs. 340 ± 225 ng/mL, P = 0·039) compared to healthy subjects. Diastolic BP z-scores were associated with disease duration, E-selectin and triglyceride levels in the T1DM group (P < 0·05). E-selectin was also related to triglycerides, otherwise there were no relationships between vascular function, markers and BP. CONCLUSION: E-selectin, an early atherosclerosis biomarker, is positively associated with diastolic BP values in children with T1DM, despite relatively short disease duration.


Subject(s)
Blood Pressure/physiology , Cardiovascular Diseases/blood , Diabetes Mellitus, Type 1/blood , E-Selectin/blood , Adolescent , Biomarkers/blood , Biomarkers/metabolism , Body Mass Index , Cardiovascular Diseases/complications , Case-Control Studies , Child , Diabetes Mellitus, Type 1/complications , Endothelium, Vascular/metabolism , Female , Humans , Hypertension/blood , Hypertension/metabolism , Intercellular Adhesion Molecule-1/blood , Male , Risk Factors , Time Factors , Triglycerides/blood , Triglycerides/metabolism , Vascular Cell Adhesion Molecule-1/blood
13.
Int J Pediatr Obes ; 6(2-2): e603-10, 2011 Jun.
Article in English | MEDLINE | ID: mdl-21142752

ABSTRACT

OBJECTIVE: We previously demonstrated beneficial effects of physical activity on cardiovascular disease (CVD) risk factors, body mass index (BMI) and fat mass in pre-pubescent obese children. The aim of this study was to determine whether these changes were maintained 2 years later. METHODS: Two years after the Randomised Controlled Trial, we performed a follow-up study with 20 of 38 subjects (11.4 ± 1.8 years). Outcomes included blood pressure (BP) by ambulatory monitoring; arterial function and structure using high-resolution ultrasound, BMI, body composition by dual-energy x-ray absorptiometry (DXA), physical activity using accelerometer, and biological markers. RESULTS: During the 2-year follow-up period, mean 24-hour diastolic BP z-score significantly decreased (1.4 ± 1.2 vs. 0.3 ± 1.4, p = 0.04), while systolic BP z-score was slightly reduced (2.4 ± 1.5 vs. 1.4 ± 1.7, p = 0.067). Blood pressure changes were greater in children who diminished their BMI z-score compared with the ones who did not. Systolic hypertension rates dropped from 50 to 28% and diastolic hypertension from 42 to 6%. In addition, arterial intima-media thickness (0.51 ± 0.03 vs. 0.51 ± 0.06, p = 0.79), BMI z-score (2.9 ± 0.8 vs. 2.9 ± 1.1 kg.cm(-2), p = 0.27), body fat (41.9 ± 6.9 vs. 42.8 ± 6.7%; p = 0.39) and physical activity count (703 ± 209 vs. 574 ± 244 cpm, p = 0.30) were stable. CONCLUSION: To our knowledge, this is the first study reporting that beneficial effects on adiposity and CVD risk factors of a physical activity centred intervention are sustained 2 years after the cessation of training in obese children. Subjects stabilized BMI z-score and maintained physical activity with further improvement of BP and stabilization of arterial wall remodelling. We conclude that it is important to encourage physical activity in this population. Effects of Aerobic Exercise Training on Arterial Function and Insulin Resistance Syndrome in Obese Children: A Randomised Controlled Trial: NCT00801645.


Subject(s)
Cardiovascular Diseases/prevention & control , Exercise Therapy , Obesity/therapy , Absorptiometry, Photon , Actigraphy/instrumentation , Adiposity , Adolescent , Age Factors , Analysis of Variance , Arteries/diagnostic imaging , Arteries/physiopathology , Blood Pressure , Blood Pressure Monitoring, Ambulatory , Body Mass Index , Cardiovascular Diseases/diagnosis , Cardiovascular Diseases/etiology , Cardiovascular Diseases/physiopathology , Chi-Square Distribution , Child , Exercise Test , Female , Follow-Up Studies , Humans , Linear Models , Male , Motor Activity , Obesity/complications , Obesity/diagnosis , Obesity/physiopathology , Oxygen Consumption , Physical Fitness , Risk Assessment , Risk Factors , Switzerland , Time Factors , Treatment Outcome , Ultrasonography
15.
J Pediatr ; 157(4): 533-9, 2010 Oct.
Article in English | MEDLINE | ID: mdl-20826281

ABSTRACT

OBJECTIVES: To measure preclinical noninvasive markers of atherosclerosis in youth with type 1 diabetes (T1DM), and to determine their associations between physical activity level and cardiorespiratory fitness (maximal oxygen consumption [VO2max]). STUDY DESIGN: This was a cross-sectional study including 32 patients with T1DM and 42 healthy subjects aged 6 to 17 years. Main outcome measures included arterial flow-mediated dilation (FMD) and intima-media thickness with high-resolution ultrasonography; physical activity by accelerometer (valid 26 patients with T1DM, 35 healthy subjects) and VO2max. RESULTS: Compared with healthy control subjects, patients with T1DM had higher intima-media thickness (mean 0.50 mm [0.48-0.52, 95% CI] vs 0.48 [0.47-0.49], P=.02) and reduced FMD (4.9% [4.1%-5.7%] vs 7.3 [6.4-8.1], P=.001), VO2max (45.5 mL/kg/min [43.0-48.0] vs 48.7 [46.7-50.6], P

Subject(s)
Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/prevention & control , Diabetes Mellitus, Type 1/epidemiology , Motor Activity , Nitroglycerin/therapeutic use , Vasodilator Agents/therapeutic use , Adolescent , Biomarkers/blood , Child , Coronary Artery Disease/blood , Cross-Sectional Studies , Endothelium, Vascular/diagnostic imaging , Exercise , Female , Health Status , Humans , Male , Tunica Intima/pathology , Ultrasonography
16.
J Thorac Cardiovasc Surg ; 140(5): 1110-6, 2010 Nov.
Article in English | MEDLINE | ID: mdl-20850141

ABSTRACT

OBJECTIVES: Acceptable coaptation cannot always be obtained using standard repair techniques. We assessed the safety and mid-term results using a novel technique to address leaflet retraction or tethering in children with type III mitral or tricuspid regurgitation as an addition to standard valve repair techniques. METHODS: Forty children were included, 36 for the mitral valve and 4 for the tricuspid valve, with a mean age of 11.3 ± 3.9 years. A polypropylene suture was placed on the free edge of the retracted or tethered leaflet segment and anchored to the atrial side of the opposite annulus. This avoided valve replacement in all patients. An additional 40 children were matched for age, etiology, leaflet retraction or tethering, and surgery in which the suspension stitch was not used and constituted the control group. RESULTS: The mean aortic crossclamp and cardiopulmonary bypass time was 36 ± 9 and 57 ± 9 minutes, respectively. No early or late deaths occurred. At discharge, no patient had more than mild regurgitation with a gradient of 4.4 ± 2.4 mm Hg in the mitral position and 2 ± 1.75 mm Hg in the tricuspid position. The results were not significantly different than those of the control group. During a follow-up of 37.7 ± 18.4 months, 3 patients required reoperation for mitral valve replacement in the suspension stitch group and 2 within the control group. At echocardiography of the remaining patients, the repair remained stable, with no suspension suture breakage. CONCLUSIONS: This suspension technique improved coaptation and resulted in avoidance or delay of valve replacement in patients with type III regurgitation, with an acceptable transvalvular gradient in most patients that did not significantly increase with growth.


Subject(s)
Cardiac Surgical Procedures , Mitral Valve Insufficiency/surgery , Suture Techniques , Tricuspid Valve Insufficiency/surgery , Adolescent , Cardiac Surgical Procedures/adverse effects , Cardiopulmonary Bypass , Case-Control Studies , Child , Constriction , Echocardiography, Doppler, Color , Female , Heart Valve Prosthesis Implantation , Hemodynamics , Humans , Kaplan-Meier Estimate , Male , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/physiopathology , Proportional Hazards Models , Reoperation , Retrospective Studies , Risk Assessment , Risk Factors , Suture Techniques/adverse effects , Switzerland , Time Factors , Treatment Outcome , Tricuspid Valve Insufficiency/diagnostic imaging , Tricuspid Valve Insufficiency/physiopathology
17.
J Thorac Cardiovasc Surg ; 140(4): 836-44, 2010 Oct.
Article in English | MEDLINE | ID: mdl-20659746

ABSTRACT

OBJECTIVE: Aortic valve repair has encouraging midterm results in selected patients. However, neither the long-term results of cusp extension nor the durability of different pericardial fixation techniques has been reported. Our goal was to address these issues. METHODS: Seventy-eight children with severe rheumatic aortic regurgitation (mean age 12 ± 3.5 years) underwent aortic valve repair using cusp extension over a 15-year period, with fresh autologous pericardium in 53 (67.9%), glutaraldehyde-fixed bovine pericardium in 9 (11.5%), and PhotoFix bovine pericardium (Sorin CarboMedics, Milano, Italy) in 16 (20.5%). Fifty-seven children (73.1%) underwent concomitant mitral valve repair, and 8 children (10.3%) underwent tricuspid valve repair. RESULTS: There was 1 operative death from left ventricular failure. During a median follow-up of 10.7 years (range 1 month to 16.4 years), 1 late death occurred and 15 patients (19.7%) required reoperation at a mean of 43 ± 33.7 months (range 1 month to 9 years), 9 within the autologous pericardium group (18%), 3 within the bovine pericardium group (33%), and 3 within the PhotoFix pericardium group (19%). Freedom from reoperation was 96% ± 2.3% at 1 year, 87.5% ± 3.9% at 5 years, 80.7% ± 4.9% at 10 years, and 75.3% ± 6% at 15 years, and was significantly decreased in the bovine pericardium group (P = .039). On multivariable analysis, greater age (hazard ratio 1.25, P < .001) and acute rheumatic carditis (hazard ratio 8.15, P = .001) at operation were significant predictors of reoperation. CONCLUSIONS: Aortic cusp extension provides adequate valve repair in a large proportion of children with rheumatic aortic regurgitation. Fresh autologous and PhotoFix pericardium trended toward better durability than glutaraldehyde-fixed bovine pericardium.


Subject(s)
Aortic Valve Insufficiency/surgery , Aortic Valve/surgery , Heart Valve Prosthesis Implantation , Rheumatic Heart Disease/surgery , Adolescent , Animals , Aortic Valve/diagnostic imaging , Aortic Valve/physiopathology , Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve Insufficiency/mortality , Aortic Valve Insufficiency/physiopathology , Bioprosthesis , Cattle , Chi-Square Distribution , Child , Disease-Free Survival , Female , Fixatives , Glutaral , Heart Valve Prosthesis , Heart Valve Prosthesis Implantation/adverse effects , Heart Valve Prosthesis Implantation/instrumentation , Heart Valve Prosthesis Implantation/mortality , Humans , Kaplan-Meier Estimate , Male , Pericardium/transplantation , Proportional Hazards Models , Recovery of Function , Reoperation , Retrospective Studies , Rheumatic Heart Disease/diagnostic imaging , Rheumatic Heart Disease/mortality , Rheumatic Heart Disease/physiopathology , Risk Assessment , Risk Factors , Time Factors , Tissue Fixation , Transplantation, Autologous , Treatment Outcome , Ultrasonography , Ventricular Function, Left , Young Adult
18.
Ann Thorac Surg ; 90(1): 317-9, 2010 Jul.
Article in English | MEDLINE | ID: mdl-20609816

ABSTRACT

Although no-patch repair was the first surgical treatment for complete atrioventricular canal, patch repairs are currently more widely used. We assessed the safety of forgoing a patch during the correction of complete atrioventricular canal in 8 consecutive patients. The complete atrioventricular canal was repaired using sutures placed on the right of the ventricular septal defect crest, passed through the bridging leaflet, and to the facing part of the ostium primum defect. There were no early deaths; all patients were in sinus rhythm without left ventricular outflow tract obstruction. This no-patch technique produces results comparable with the modified single-patch repair, while reducing ischemic time.


Subject(s)
Endocardial Cushions/surgery , Child, Preschool , Humans , Infant
19.
Int J Pediatr Obes ; 5(3): 230-6, 2010 May 03.
Article in English | MEDLINE | ID: mdl-20210676

ABSTRACT

BACKGROUND: Obstructive sleep apnoea (OSA) in obese adults is associated with cardiovascular disease independently of obesity. Vascular alterations exist in children with obesity and may constitute the first stage in the development of adulthood cardiovascular disease. OBJECTIVE: To investigate the relationship between OSA and early arterial alterations in obese children. DESIGN: Cross-sectional study of a prospective cohort. PATIENTS: A total of 51 children with severe obesity managed at a teaching hospital outpatient clinic. MEASUREMENTS: Polysomnography was performed. We measured the intima-media thickness and incremental elastic modulus (Einc) to assess the mechanical characteristics of the common carotid artery. Arterial endothelial function was evaluated by measuring flow-mediated dilation and glyceryl trinitrate-mediated dilation (GTNMD) of the brachial artery. RESULTS: A total of 24 (47%) children had a desaturation index (DI) >10/h and 7 (14%) had a respiratory event index >10/h. DI >10/h was associated with significantly higher values of Einc (4.0 + or - 0.5 vs. 2.4 + or - 0.4 mm Hg(-1) x 10(3), p=0.003) and GTNMD (18.0 + or - 1.1 vs. 14.1 + or - 1.0 %, p=0.02) after adjustment for age, sex, body mass index, fasting insulin, and leptin. In the univariate analysis, GTNMD correlated positively with DI (r=0.14, p=0.02) after adjustment for age, sex, fasting insulin and leptin. By multivariate analysis with BMI as an additional independent variable, both GTNMD and Einc correlated significantly with DI (beta=0.4, p=0.02 and beta=0.27, p=0.04, respectively). CONCLUSIONS: OSA in children is associated with arterial alterations independently from obesity. The increased vasodilation in response to glyceryl trinitrate reflects pre-existing vasoconstriction probably induced by intermittent hypoxia. OSA should be detected early in children with severe obesity.


Subject(s)
Cardiovascular Diseases/epidemiology , Cardiovascular Diseases/physiopathology , Obesity/epidemiology , Obesity/physiopathology , Sleep Apnea, Obstructive/epidemiology , Biomechanical Phenomena , Brachial Artery/physiopathology , Child , Cross-Sectional Studies , Female , Humans , Hypoxia/epidemiology , Hypoxia/physiopathology , Male , Polysomnography , Severity of Illness Index , Sleep Apnea, Obstructive/diagnosis , Vasoconstriction
20.
Arch Cardiovasc Dis ; 103(1): 10-8, 2010 Jan.
Article in English | MEDLINE | ID: mdl-20142115

ABSTRACT

BACKGROUND: Many studies performed in adults have reported the involvement of genetic determinants in vascular alterations that predispose to cardiovascular diseases later in life. To date, no study has assessed the co-involvement of gene polymorphisms as cardiovascular risk factors in children. AIMS: To search for variants involved in early vascular alterations in obese children. METHODS: In 232 obese children, we performed an association study between variables related to endothelial function or arterial mechanical properties and functional variants reported to predispose towards vascular alterations in adults. Candidate polymorphisms were selected in genes involved in the renin-angiotensin system, vascular endothelial cell remodelling and communication, arterial inflammation, adiponectin production and lipoprotein metabolism. Non-invasive arterial measurements were performed to evaluate the mechanical characteristics of the common carotid artery and the endothelial function of the brachial artery. RESULTS: We found no association between the polymorphisms studied, taken alone or in combination with the arterial variables measured. CONCLUSION: Our hypothesis predicting that the tested genetic variants, which are involved in adult cardiovascular diseases, may influence the susceptibility to early vascular alterations in obese children was not validated. Thus, obesity-associated metabolic complications appear to remain the main predictors of arterial alterations in obese children.


Subject(s)
Obesity/genetics , Polymorphism, Genetic , Vascular Diseases/genetics , Adolescent , Brachial Artery/physiopathology , Carotid Artery, Common/physiopathology , Child , Compliance , Endothelium, Vascular/physiopathology , Female , Gene Frequency , Genetic Association Studies , Genetic Predisposition to Disease , Humans , Logistic Models , Male , Obesity/complications , Obesity/diagnostic imaging , Obesity/physiopathology , Phenotype , Prospective Studies , Risk Assessment , Risk Factors , Ultrasonography , Vascular Diseases/diagnostic imaging , Vascular Diseases/physiopathology , Vasodilation
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