Congenital cardiac surgery

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Heart transplantation using a donor with partial anomalous pulmonary venous connection and atrial septal defect.

Jue, 04/06/2017 - 19:52
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Heart transplantation using a donor with partial anomalous pulmonary venous connection and atrial septal defect.

Interact Cardiovasc Thorac Surg. 2017 Mar 30;:

Authors: de Beco G, Duisit J, Poncelet AJ

Abstract
Over the last decade, the shortage of donors has led to increased waiting time prior to transplantation and its related mortality. Therefore, extended criteria for donor hearts have been proposed. In this report, we describe a successful transplantation despite a diagnosis of partial abnormal pulmonary venous return associated with an atrial septal defect sinus venosus and persisting left-sided superior vena cava. Knowledge in congenital cardiac disease can broaden the definition of 'marginal' donor hearts and allow their use without increasing the risk of transplantation.

PMID: 28379508 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

Diagnosis, imaging and clinical management of aortic coarctation.

Jue, 04/06/2017 - 19:52
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Diagnosis, imaging and clinical management of aortic coarctation.

Heart. 2017 Apr 04;:

Authors: Dijkema EJ, Leiner T, Grotenhuis HB

Abstract
Coarctation of the aorta (CoA) is a well-known congenital heart disease (CHD), which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of CoA remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (>20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV). Transthoracic echocardiography is the primary imaging modality for suspected CoA. However, cardiac magnetic resonance imaging is the preferred advanced imaging modality for non-invasive diagnosis and follow-up of CoA. Adequate and timely diagnosis of CoA is crucial for good prognosis, as early treatment is associated with lower risks of long-term morbidity and mortality. Numerous surgical and transcatheter treatment strategies have been reported for CoA. Surgical resection is the treatment of choice in neonates, infants and young children. In older children (>25 kg) and adults, transcatheter treatment is the treatment of choice. In the current era, patients with CoA continue to have a reduced life expectancy and an increased risk of cardiovascular sequelae later in life, despite adequate relief of the aortic stenosis. Intensive and adequate follow-up of the left ventricular function, valvular function, blood pressure and the anatomy of the heart and the aorta are, therefore, critical in the management of CoA. This review provides an overview of the current state-of-the-art clinical diagnosis, diagnostic imaging algorithms, treatment and follow-up of patients with CoA.

PMID: 28377475 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

The therapeutic characteristics of serial casting on congenital scoliosis: a comparison with non-congenital cases from a single-center experience.

Jue, 04/06/2017 - 19:52
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The therapeutic characteristics of serial casting on congenital scoliosis: a comparison with non-congenital cases from a single-center experience.

J Orthop Surg Res. 2017 Apr 04;12(1):56

Authors: Cao J, Zhang XJ, Sun N, Sun L, Guo D, Qi XY, Bai YS, Sun BS

Abstract
BACKGROUND: The therapeutic efficacy of serial casting on idiopathic scoliosis has been gradually documented. However, literatures on serial casting for congenital scoliosis (CS) remain extremely rare. This paper aimed to compare the treatment outcomes of serial casting between CS and non-CS patients to comprehensively evaluate the therapeutic characteristics of serial casting on CS patients.
METHODS: A total of 23 early-onset scoliosis cases were included and divided into congenital scoliosis (CS, n = 8) and non-congenital group (non-CS, n = 15). Therapeutic outcomes including the major curve Cobb angle, thoracic kyphosis angle, lumbar lodosis angle, and thoracic spine growing rate were compared between groups at precast, after the first cast, and at the latest follow-up, respectively.
RESULTS: All patients received the first cast at the age of 3.25 ± 1.20 years and 5.70 ± 1.18 times of cast corrections. The average casting time was 17.17 ± 3.38 months, and the mean follow-up time was 23.91 ± 12.28 months. Both CS and non-CS groups had significant decrease in Cobb angle after the first cast and at the latest follow-up (all P < 0.05). Cobb angle was significantly lower in non-CS group than in CS group at both time points (all P < 0.01). The correction rate of Cobb angle was significantly higher in non-CS group than in CS group (around 50 vs. 20%, both P < 0.01). The mean thoracic growth rate was significantly lower in CS group than in non-CS group (0.72 ± 0.20 vs. 1.42 ± 0.22 cm/year, P < 0.001). At the latest follow-up, there are 2 cases receiving growing rod surgery, 8 cases wearing a brace, and 13 cases continuing serial casting.
CONCLUSIONS: Although the therapeutic efficacy of casting on CS patients is not as good as that on non-CS patients, casting is still an efficient treatment option for CS patients to delay the need for initial surgery.

PMID: 28376819 [PubMed - in process]

Categorías: Cirugía congénitos

Insertion of a totally implantable venous access port in a patient with persistent left superior vena cava (PLSVC).

Jue, 04/06/2017 - 19:52
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Insertion of a totally implantable venous access port in a patient with persistent left superior vena cava (PLSVC).

Acta Chir Belg. 2017 Apr 05;:1-4

Authors: Van Walleghem J, Depuydt S, Schepers S

Abstract
Persistent left superior vena cava (PLSVC) is a rare congenital variant, but it is the most common venous thoracic malformation, occurring in 0.3-0.5% of the general population. PLSVC is caused by the persistence of the embryological left anterior cardinal vein which normally obliterates and persists as the Marshall ligament. We present the case of a 74-year-old male lung cancer patient, who already underwent an uncomplicated right-hand sided pacemaker insertion in 2006. During implantation of a subcutaneous central venous access port through puncture of the left subclavian vein, a PLSVC was discovered. A venography and computed tomography confirmed the anomaly. The catheter of the central venous access port was positioned in the PLSVC with adequate drainage and flushing of blood, rendering it useful for administration of systemic chemotherapy. The patient underwent his chemotherapy cycles without major complications.

PMID: 28376682 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

Successful endovascular coil embolization in an elder and asymptomatic case of anomalous systemic arterial supply to the normal basal segment.

Mié, 04/05/2017 - 18:48
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Successful endovascular coil embolization in an elder and asymptomatic case of anomalous systemic arterial supply to the normal basal segment.

Int J Surg Case Rep. 2017 Mar 22;34:103-105

Authors: Machida Y, Motono N, Matsui T, Usuda K, Uramoto H

Abstract
INTRODUCTION: An anomalous systemic arterial supply to the normal basal segment without sequestration is a rare congenital vascular malformation. The discovery age is relatively young, and the most common clinical symptom is hemoptysis due to pulmonary hypertension or heart failure. We herein describe a case of endovascular embolization of in an elderly and asymptomatic patient with an anomalous systemic arterial supply to the normal basal segment.
PRESENTAITION OF CASE: An 80-year-old male was referred to our hospital due to an abnormal chest shadow. The patient was diagnosed with an anomalous systemic arterial supply to normal basal segment. We performed coil embolization via the catheterization.
DISCUSSION: The application of coil embolization via catheterization results in a low risk of infection and small burden on the body compared with surgery. There are few report of the coil embolization for an anomalous systemic arterial supply to the normal basal segment. Hence, it is necessary to accumulate additional cases.
CONCLUSION: The outcome of thie case indicates that coil embolization is a very useful treatment method for elderly patients with an anomalous systemic arterial supply to the normal basal segment.

PMID: 28376417 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

The Cost Differential Between Warfarin Versus Aspirin Treatment After a Fontan Procedure.

Mié, 04/05/2017 - 18:48
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The Cost Differential Between Warfarin Versus Aspirin Treatment After a Fontan Procedure.

Heart Lung Circ. 2017 Mar 06;:

Authors: Schilling C, Dalziel K, Iyengar AJ, d'Udekem Y

Abstract
BACKGROUND: The use of aspirin versus warfarin for treatment of patients after a Fontan procedure remains contentious. Current preference-based models of treatment across Australia and New Zealand show variation in care that is unlikely to reflect patient differences and/or clinical risk.
METHODS: We combine data from the Australian and New Zealand Fontan Registry and a home INR (International Normalised Ratio) monitoring program (HINRMP) from the Royal Children's Hospital (RCH) Melbourne, to estimate the cost difference for Fontan recipients receiving aspirin versus warfarin for 2015. We adopt a societal perspective to costing which includes cost to the health system (e.g. medical consults, pathology tests) and costs to patients and carers (e.g. travel and time), but excludes costs of adverse events. Costs are presented in Australian 2015 dollars; any costs from previous years have been inflated using appropriate rates from the Australian Bureau of Statistics.
RESULTS: We find that warfarin patients face additional costs of $825 per annum, with the majority ($584 or 71%) of those borne by the patient or family. If aspirin is as clinically as effective as warfarin, Fontan recipients could be enjoying far less costly, invasive and time-consuming treatment. While achieving such clinical consensus can be difficult, economics shows us that there are large costs associated with a failure to achieve it.

PMID: 28372885 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

Non-invasive cardiac output monitor validation study in pediatric cardiac surgery patients.

Mié, 04/05/2017 - 18:48
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Non-invasive cardiac output monitor validation study in pediatric cardiac surgery patients.

J Clin Anesth. 2017 May;38:129-132

Authors: Tirotta CF, Lagueruela RG, Madril D, Velis E, Ojito J, Monroe D, Aguero D, Irizarry M, McBride J, Hannan RL, Burke RP

Abstract
STUDY OBJECTIVE: To validate a non-invasive cardiac output monitor in pediatric cardiac surgery patients.
DESIGN: Prospective trial.
SETTING: Operating room.
PATIENTS: 20 pediatric cardiac surgery patients ASA physical status 3 and 4.
INTERVENTIONS: Aesculon noninvasive cardiac output monitor was used and compared to the cardiac output derived from the Fick equation. Oxygen consumption was measured and blood samples were taken from the arterial line and from the superior and inferior vena cava.
MEASUREMENTS: Noninvasive cardiac output and cardiac index and Fick cardiac output and cardiac index. Oxygen consumption was measured by the TreyMed metabolic monitor. Blood samples were simultaneously drawn from the arterial line and from the superior and inferior vena cava purse string sites by the surgeon, prior to commencing cardiopulmonary bypass. Another data set was obtained right after termination of cardiopulmonary bypass.
RESULTS: There was a direct, significant relationship between Fick CO/CI and NICOM CO/CI measurements. More dispersion is detected when the magnitude of the measure increases, i.e., for older and larger patients.
CONCLUSIONS: There is a strong correlation between the cardiac output values derived from the Fick equation and the Aesculon non-invasive cardiac output monitor.

PMID: 28372651 [PubMed - in process]

Categorías: Cirugía congénitos

Prenatally diagnosed congenital portosystemic shunts.

Mié, 04/05/2017 - 18:48
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Prenatally diagnosed congenital portosystemic shunts.

J Matern Fetal Neonatal Med. 2017 Apr 03;:1-14

Authors: Francois B, Lachaux A, Gottrand F, De Smet S

Abstract
AIM: Congenital portosystemic shunts (CPSS) are rare, congenital malformations that are increasingly often discovered during the fetal period, and for which the manifestations and evolution are poorly understood. The objective of this review is to describe the phenotype and evolution of forms diagnosed in the antenatal period.
MATERIALS AND METHODS: We performed a systematic review of the literature cited in Pubmed between 1982 and 2016 for CPSS cases diagnosed during the fetal period.
RESULTS: We identified 123 cases. The median age at diagnosis was 25 GA (14 - 38 weeks GA). Eighty patients had 128 associated congenital anomalies. The congenital abnormalities most frequently associated with antenatal diagnosis of CPSS were congenital cardiac disease (30 cases), intrauterine growth restriction (21 cases), vascular anomalies (14 cases) and trisomy 21 (7 cases). Seventy-five complications were reported in the literature. The most frequent were antenatal hemodynamic abnormalities (27 cases), neonatal cholestasis (11 cases) and hyperammonemia (10 cases). Twenty-nine patients had no complications. The choice of treatment was conservative in 29/56 cases, interventional radiology in 15 cases and surgery in 15 cases (3 of the latter after failure of embolization).
CONCLUSION: From this review we propose an algorithm for the perinatal management of this congenital abnormality.

PMID: 28372492 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

Clinically apparent long-term electric disturbances in the acute and very long-term of patent foramen ovale device-based closure.

Mié, 04/05/2017 - 18:48
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Clinically apparent long-term electric disturbances in the acute and very long-term of patent foramen ovale device-based closure.

Cardiovasc Revasc Med. 2017 Mar;18(2):118-122

Authors: Rigatelli G, Zuin M, Pedon L, Zecchel R, Dell'Avvocata F, Carrozza A, Zennaro M, Pastore G, Zanon F

Abstract
BACKGROUND/PURPOSE: Incidence of electrical disturbances in patients submitted to transcatheter patent foramen ovale (PFO) closure has not been fully clarified in a large population. The aim of the study is to assess the incidence of atrial fibrillation, supraventricular tachi-arrhythmias, and atrio-ventricular block in the acute and very long-term follow-up.
METHODS/MATERIALS: We reviewed the medical and instrumental data of 1000 consecutive patients (mean age 47.3±17.1years) prospectively enrolled in two centers over a 13-year period (February 1999 to February 2012) for right-to-left (R-to-L) shunt ICE-aided catheter-based closure using different devices.
RESULTS: Successful transcatheter PFO closure was achieved in 99.8% of the patients. Implanted devices were: Amplatzer PFO Occluder in 463 patients (46.3%), Amplatzer ASD Cribriform Occluder in 420 patients (42.0%), Premere Occlusion System in 95 patients (9.5%), and Biostar Occluder in 22 patients (2.2%). Postprocedural electrical complications occurred in 5.9% of patients. The only independent predictors of electrophysiological complications were female gender (OR 2.3, 0.5-5.1 [95% CI], p<0.001) and device disk >30mm (OR 5.0, 1.2-7.2 [95% CI], p<0.001). On a mean follow-up of 12 .3±0.6years (minimum 4- maximum 17years), electrical complications occurred in 1.4% of patients including one only case of complete AVB and 5 cases of permanent AF. The only independent predictors were female gender (OR 2.3, 0.5-5.1 [95% CI], p<0.001) and device disk >30mm (OR 5.0, 1.2-7.2 [95% CI], p<0.001).
CONCLUSION: Device-based closure of PFO using different devices, appeared very safe from an electrophysiological point of view with low incidence of electrical disturbances even in the very long-term follow-up.

PMID: 27847261 [PubMed - indexed for MEDLINE]

Categorías: Cirugía congénitos

Diverse multi-organ histopathologic changes in a failed Fontan patient.

Mié, 04/05/2017 - 18:48
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Diverse multi-organ histopathologic changes in a failed Fontan patient.

Pediatr Int. 2016 Oct;58(10):1061-1065

Authors: Mizuno M, Ohuchi H, Matsuyama TA, Miyazaki A, Ishibashi-Ueda H, Yamada O

Abstract
We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension. No major ulcerative lesions were found in the small or large intestines. Dilated lymph ducts, one of the characteristic features of PLE, were not seen in mucosal and submucosal areas. Liver cirrhosis was obvious despite little increase in liver enzymes. Histological changes in bilateral kidneys were subtle despite repeated episodes of renal failure. Thus, there may be significant discrepancies between clinical manifestations and multi-organ histological changes in failed Fontan patients.

PMID: 27616284 [PubMed - indexed for MEDLINE]

Categorías: Cirugía congénitos

[Comparative results of Fontan surgery in patients with and without hypoplastic left heart syndrome].

Mié, 04/05/2017 - 18:48
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[Comparative results of Fontan surgery in patients with and without hypoplastic left heart syndrome].

Rev Chil Pediatr. 2016 Sep - Oct;87(5):366-372

Authors: Becker Rencoret P, Besa Bandeira S, Riveros González S, Frangini Sanhueza P, Springmüller Pinto D, González Foretic R, Urcelay Montecinos G

Abstract
INTRODUCTION: During the last few years, numerous patients with univentricular heart disease have been treated surgically with total cavopulmonary anastomosis according to a staged surgery protocol in our institution.
OBJECTIVE: To evaluate the perioperative outcomes and survival of patients with hypoplastic left heart syndrome (HLHS) after the Fontan procedure and compare them with other types of univentricular heart disease.
PATIENTS AND METHOD: A total of 102 patients underwent a Fontan procedure between April 1996 and March 2014, 25 with HLHS (group I), and 77 patients with other types of univentricular heart disease (group II). Groups survival, demographics, hemodinamic studies, morbimortality, mechanical ventilation, surgical drains, post-operative stay, isotopes score, pacemaker use, and requiriment of Fontan takedown were analyzed.
RESULTS: Intraoperative mortality was 4% (n=1) for group I, and 7.8% (n=6) for group II (P=.451). A difference was only found in hospital length of stay (LOS), being 17 days (6-47) for group I and 12 days (5-103) for group II (P=.017). Mean follow-up was 4.24±2.08 years for group I, and 8.7±4.67 for group II. Survival rate at 8 years for both groups was 88%, and 81% at 10 years for group II.
CONCLUSIONS: The Fontan procedure had similar mortality, but longer LOS, in patients with HLHS compared to those with another types of single ventricle anatomy. Long term survival was comparable between both groups.

PMID: 27091396 [PubMed - indexed for MEDLINE]

Categorías: Cirugía congénitos

[Hospital morbidity and mortality in children with Down's syndrome: Experience in a university hospital in Chile].

Mié, 04/05/2017 - 18:48
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[Hospital morbidity and mortality in children with Down's syndrome: Experience in a university hospital in Chile].

Rev Chil Pediatr. 2016 Mar-Apr;87(2):102-9

Authors: Lizama Calvo M, Cerda Lorca J, Monge Iriarte M, Carrillo Mayanquer I, Clavería Rodríguez C, Castillo Moya A

Abstract
INTRODUCTION: Children with Down's syndrome (DS) have a higher risk of congenital malformations and acute diseases, with increased risk of hospital admissions compared with the general population. This study describes patterns of hospital admissions for children and adolescents with DS.
PATIENTS AND METHODS: A retrospective study of hospital admissions of children with DS, younger than 15 years old, and cared for by the Paediatric Department of the Hospital Clínico Pontificia Universidad Católica de Chile, between 2008 and 2011.
RESULTS: There were 222 admissions of 161 patients with DS during the study period, of which 110 were girls. The median age was 8 months, and the median hospital stay was 6 days. Just over half (56.7%) of the hospital stays were in the Paediatric Critic Care Unit. Heart surgery was performed on 59.4%, and the principal congenital heart defect attended was atrioventricular canal. The principal diagnosis, other than heart surgery, was lower respiratory tract infection. In this series, 3 children died.
CONCLUSIONS: Children with DS are a relevant group for inpatient care, because their high incidence in Chile, their respiratory and cardiovascular risk, prolonged hospitalizations, high frequency of critical care days and mortality risk. This group has special and complex needs during their hospitalizations and it is necessary to create a multidisciplinary team with competences to take care the particular characteristics of this vulnerable group.

PMID: 26627695 [PubMed - indexed for MEDLINE]

Categorías: Cirugía congénitos

[Hypoplastic left heart syndrome: 10 year experience with staged surgical management].

Mié, 04/05/2017 - 18:48
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[Hypoplastic left heart syndrome: 10 year experience with staged surgical management].

Rev Chil Pediatr. 2016 Mar-Apr;87(2):121-8

Authors: Urcelay G, Arancibia F, Retamal J, Springmuller D, Clavería C, Garay F, Frangini P, González R, Heusser F, Arretz C, Zelada P, Becker P

Abstract
UNLABELLED: Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country.
OBJECTIVES: 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality.
PATIENTS AND METHOD: Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed.
RESULTS: Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤2mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P=.05). Actuarial survival was 64% at one year, and 57% at 5years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality.
CONCLUSIONS: Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

PMID: 26455701 [PubMed - indexed for MEDLINE]

Categorías: Cirugía congénitos

Convex Hemiepiphysiodesis: Posterior/anterior in-situ Versus Posterior-only With Pedicle Screw Instrumentation: An Experimental Simulation in Immature Pigs.

Mié, 04/05/2017 - 18:48
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Convex Hemiepiphysiodesis: Posterior/anterior in-situ Versus Posterior-only With Pedicle Screw Instrumentation: An Experimental Simulation in Immature Pigs.

J Pediatr Orthop. 2016 Dec;36(8):847-852

Authors: Bekmez S, Demirkiran HG, Yilmaz G, Akel I, Atilla P, Muftuoglu SF, Yazici M, Alanay A

Abstract
STUDY DESIGN: Experimental study.
BACKGROUND: Convex growth arrest (CGA) has been commonly used in the treatment of long-sweeping congenital deformities of the immature spine. As there are major drawbacks about the anterior procedure in the conventional CGA method, a new modification has been documented that using only posterior spinal approach with pedicle screw instrumentation. The aim of the study was to compare posterior-only CGA using pedicle screws with combined anterior/posterior in-situ CGA for the findings in histologic, radiologic, and manual palpation examinations in an immature pig model.
METHODS: Twelve 10-weeks old pigs were grouped into 2. In group 1, posterior-only, pedicle screw instrumented CGA was performed on the left side of L1-L4 vertebrae. In group 2, conventional combined posterior and anterior CGA was performed to the left side of L1-L4 vertebrae without instrumentation. All animals were killed twelve weeks after surgery. T11-L5 segments were en-bloc resected and radiologic, histologic, and manual palpation examinations were done.
RESULTS: Marked scoliotic (12.2±2.5 and 9.2±1.3 in group 1 and 2, respectively) and kyphotic (11.2±1.0 degrees for the group 1 and 12±5.2 degrees for the group 2, respectively) deformities were noted in both groups, which were caused by hemiepiphysiodesis effect. Anterior and posterior parts of group 2 and posterior part of group 1 demonstrated fusion in histologic and radiologic analyzes. In anterior part of the group 1, marked narrowing on the disk spaces and thinning of growth plates were noted in radiologicg examination, chondrocyte degeneration, and newly-formed bone trabeculae in disk-space were noted in histological examination. In manual palpation, no motion was detected in group 1 and motion was detected in only one segment of one animal in group 2.
CONCLUSIONS: Anterior growth of the vertebrae can be controlled by application of posterior transpedicular screws and rod. Such an effect can eliminate the need for anterior surgical intervention in convex hemiepiphysiodesis procedures.
CLINICAL RELEVANCE: The instrumented CGA technique provides a satisfactory epiphysiodesis effect both anteriorly and posteriorly, as previously demonstrated by clinical studies.

PMID: 26090986 [PubMed - indexed for MEDLINE]

Categorías: Cirugía congénitos

A multicentre study of patients with Timothy syndrome.

Mar, 04/04/2017 - 18:18
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A multicentre study of patients with Timothy syndrome.

Europace. 2017 Mar 24;:

Authors: Walsh MA, Turner C, Timothy KW, Seller N, Hares DL, James AF, Hancox JC, Uzun O, Boyce D, Stuart AG, Brennan P, Sarton C, McGuire K, Newbury-Ecob RA, Mcleod K

Abstract
Aims: Timothy syndrome (TS) is an extremely rare multisystem disorder characterized by marked QT prolongation, syndactyly, seizures, behavioural abnormalities, immunodeficiency, and hypoglycaemia. The aim of this study was to categorize the phenotypes and examine the outcomes of patients with TS.
Methods and results: All patients diagnosed with TS in the United Kingdom over a 24-year period were reviewed. Fifteen centres in the British Congenital Arrhythmia Group network were contacted to partake in the study. Six patients with TS were identified over a 24-year period (4 boys and 2 girls). Five out of the six patients were confirmed to have a CACNA1C mutation (p.Gly406Arg) and the other patient was diagnosed clinically. Early presentation with heart block, due to QT prolongation was frequently seen. Four are still alive, two of these have a pacemaker and two have undergone defibrillator implantation. Five out of six patients have had a documented cardiac arrest with three occurring under general anaesthesia. Two patients suffered a cardiac arrest while in hospital and resuscitation was unsuccessful, despite immediate access to a defibrillator. Surviving patients seem to have mild developmental delay and learning difficulties.
Conclusion: Timothy syndrome is a rare disorder with a high attrition rate if undiagnosed. Perioperative cardiac arrests are common and not always amenable to resuscitation. Longer-term survival is possible, however, patients invariably require pacemaker or defibrillator implantation.

PMID: 28371864 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes.

Mar, 04/04/2017 - 18:18
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Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes.

Prenat Diagn. 2017 Mar 31;:

Authors: Kailin J, Dhillon G, Maskatia S, Cass D, Shamshirsaz A, Mehollin-Ray A, Cassady C, Ayres N, Wang Y, Belfort M, Olutoye O, Ruano R

Abstract
OBJECTIVES: Fetuses with congenital diaphragmatic hernia (CDH) demonstrate varying degrees of left heart hypoplasia. Our study assesses the relationship between fetal left-sided cardiac structural dimensions, lung size, percentage liver herniation, lung-to-head ratio, postnatal left-sided cardiac structural dimensions, and postnatal outcomes.
METHODS: We performed a retrospective cohort study of fetuses with left-sided CDH who had prenatal echocardiographic, ultrasound and MRI examinations at our institution between January 2007 and March 2015. Postnatal outcomes assessed include use of inhaled nitric oxide (iNO), use of extracorporeal membrane oxygenation (ECMO) and death.
RESULTS: Fifty-two fetuses with isolated left-sided CDH were included. Multivariate logistic regression models indicated that smaller fetal aortic valve z-score was associated with postnatal use of iNO (p = 0.03). Fetal mitral valve z-score correlated with lung-to-head ratio (p = 0.04), postnatal mitral valve z-score correlated with percent liver herniation (p = 0.03), and postnatal left ventricular end-diastolic dimension z-score correlated with liver herniation < 20% (p = 0.04).
CONCLUSION: We identified associations between smaller fetal left-sided cardiac structural dimensions and classic CDH indices. Smaller aortic valve z-score was associated with iNO use; however, left heart dimensions showed no association with ECMO or mortality. Further study into the impact of left-sided hypoplasia on outcomes in CDH is worthy of evaluation in a larger, prospective study.

PMID: 28370263 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

Recipient Twin Circular Shunt Physiology Before Fetal Laser Surgery: Survival and Risks for Postnatal Right Ventricular Outflow Tract Obstruction.

Mar, 04/04/2017 - 18:18
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Recipient Twin Circular Shunt Physiology Before Fetal Laser Surgery: Survival and Risks for Postnatal Right Ventricular Outflow Tract Obstruction.

J Ultrasound Med. 2017 Apr 03;:

Authors: Pruetz JD, Votava-Smith JK, Chmait HR, Korst LM, Llanes A, Chmait RH

Abstract
OBJECTIVES: To evaluate risk for congenital heart disease (CHD) in recipient twins with circular shunt physiology (CSP).
METHODS: This prospective study enrolled twin-twin transfusion syndrome (TTTS) cases from 2006 to 2015. Fetal echocardiography (FE) was performed before laser surgery when cardiac involvement was suspected. Diagnosis of recipient twin CSP required tricuspid and pulmonary regurgitation, right ventricular dysfunction, and flow reversal in the ductus arteriosus. Outcomes were assessed at 30 days after birth.
RESULTS: Of the 496 TTTS pregnancies, 20 (4%) met the criteria for CSP. Among those born alive, who had documented cardiac outcomes (n = 457), patients with CSP were more likely to have CHD, specifically right ventricular outflow tract obstruction (5 of 18 [27.8%] versus 22 of 439 [5.0%], odd ratio [OR] 7.29, 95% confidence interval [CI] 2.05-24.72, P = .0025). Of the recipient twins with preoperative FE (n = 259, 52%) who were born alive and had documented cardiac outcomes (n = 242), those with CSP were still more likely to have right ventricular outflow tract obstruction (5 of 18 [27.8%] versus 14 of 224 [6.3%], OR 5.77, CI 1.54-20.92, P = .0077). With both analyses, twins with CSP had higher Quintero stage, but similar patient characteristics and 30-day mortality compared with those without CSP. Subgroup analyses of the CSP cohort identified no differences in preoperative characteristics or FE findings predictive of CHD.
CONCLUSIONS: Recipient twins with preoperative CSP were at increased risk for postnatal right ventricular outflow tract obstruction, but appeared to have comparable survival after fetal laser surgery despite these dramatic pathophysiological prenatal findings. Preoperative FE in TTTS remains important for prediction of postnatal CHD.

PMID: 28370096 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

National trends and variability in blood utilization in paediatric cardiac surgery.

Mar, 04/04/2017 - 18:18
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National trends and variability in blood utilization in paediatric cardiac surgery.

Interact Cardiovasc Thorac Surg. 2017 Mar 28;:

Authors: Karimi M, Sullivan JM, Lerer T, Hronek C

Abstract
OBJECTIVES: Red blood cell transfusion is a common practice in paediatric cardiac surgery. Transfusion of red blood cells has been shown to be associated with an increase in morbidity in paediatric patients undergoing cardiac surgery. There is a huge variability in the practice of blood utilization within and across different practices. The aim of this study was to demonstrate the current variability and the trends over the past decade in blood usage among children's hospitals performing paediatric cardiac surgery.
METHODS: We performed a retrospective observational cohort study using the Paediatric Health Information System database from 43 participating paediatric hospitals in the USA. All discharge billing information for patients younger than 19 years of age who underwent cardiac surgery using cardiopulmonary bypass between 2005 and 2015 was investigated. Ten index diagnoses and procedures were investigated and analysed, based on age differences and on the Society of Thoracic Surgeon & European Association for Cardio-Thoracic Surgery mortality scores. Our main outcome variable was the unit(s) of homologous red blood cells charged for the first 24 h of admission for cardiac surgery.
RESULTS: There was significant variability in red blood cell usage for a given diagnosis and procedural code across all the centres despite varied age ranges and complexity scores for the current and the last decade of paediatric cardiac surgical patients.
CONCLUSIONS: We found a discernible variability in the current practice of blood utilization for a given procedure despite variability in the age and complexity of patients, with no changes in practice patterns for the last decade of paediatric cardiac surgery.

PMID: 28369475 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

Significant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiogr

Mar, 04/04/2017 - 18:18
Related Articles

Significant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiography.

Eur J Cardiothorac Surg. 2017 Mar 28;:

Authors: Jia Q, Cen J, Zhuang J, Zhong X, Liu X, Li J, Liang C, Huang M

Abstract
OBJECTIVES: The prognosis of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD-MAPCAs) after surgery shows substantial clinical heterogeneity and predictors for outcomes are lacking. This study aimed to assess the predictive value of preoperative cardiac computed tomography angiography (CTA) for survival in patients with PA-VSD-MAPCAs.
METHODS: We retrospectively analysed PA-VSD-MAPCA patients with preoperative CTA who underwent both right ventricular outflow tract reconstruction and MAPCA unifocalization ( n  = 24) or pulmonary artery rehabilitation ( n  = 28). The end-point was overall survival. Prognostic values of CTA were assessed using Cox univariate and multivariate analyses. The significant threshold of independent parameters was calculated using receiver-operating characteristic (ROC) curves.
RESULTS: During a median follow-up of 1145 days, a total of 13 deaths were observed. Multivariate analysis identified a high pulmonary vein index (PVI) [hazard ratio (HR) = 0.03; 95% confidence interval (CI): 0.03, 0.28; P  <   0.01] and the presence of native pulmonary artery (HR = 0.06; 95% CI: 0.10, 0.35; P  <   0.01) as independent positive predictors of better survival. The area under the ROC curve for PVI was 0.79 ( P  <   0.01), and a cut-off point of 438 mm 2 /m 2 was deemed the significant threshold for survival (sensitivity 92%, specificity 72%).
CONCLUSIONS: Preoperational high PVI and native pulmonary artery presence were significant morphologic predictors of a positive survival advantage in PA-VSD-MAPCA patients. A PVI ≥438 mm 2 /m 2 may be a reliable positive prognosticator that could improve the decision-making strategy for PA-VSD-MAPCA patients.

PMID: 28369397 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos

Absorbable pulmonary artery banding: a strategy for reducing reoperations†.

Mar, 04/04/2017 - 18:18
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Absorbable pulmonary artery banding: a strategy for reducing reoperations†.

Eur J Cardiothorac Surg. 2017 Mar 27;:

Authors: Daley M, Brizard CP, Konstantinov IE, Brink J, Jones B, d'Udekem Y

Abstract
OBJECTIVES: Pulmonary artery banding (PAB) remains in the armamentarium of techniques for several congenital cardiac anomalies, but necessitates a reoperation for eventual removal. We sought to assess the efficacy of an absorbable PAB in obviating the requirement for reoperation.
METHODS: From 2003 to 2015, 45 consecutive patients with a median age of 1.6 months (2 days-11 months) underwent placement of a polydioxanone absorbable PAB. In 28 patients (62%), the band was placed concomitantly to a ventricular septal defect (VSD) closure and in 17 (38%), the band was the sole procedure for the VSDs. Fourteen patients had additional cardiac anomalies and 9 patients had aortic arch anomalies, which were repaired at the time of absorbable PAB placement.
RESULTS: There was 1 hospital death. The band was removed early in 3 patients. Mean time to follow-up was 5.2 ± 3.5 years. Overall, 32 of the 41 patients discharged from hospital with absorbable PABs did not require reoperation on the VSDs or the band, resulting in a single-stage definitive repair of 78%. Median time to absorbable PAB resorption was 7.2 months (interquartile range, 5.0-10.4). Freedom from reoperation related to residual VSDs or PAB obstruction was 78% (95% CI: 61-87%) at 10 years.
CONCLUSIONS: The use of absorbable polydioxanone PABs is an effective method of reducing reoperations for multiple VSDs. Their use should be incorporated in the surgical strategy for repair of muscular and residual VSDs.

PMID: 28369343 [PubMed - as supplied by publisher]

Categorías: Cirugía congénitos