Cirugía congénitos

HAND1 loss-of-function mutation contributes to congenital double outlet right ventricle.

Int J Mol Med. 2017 Jan 20;:

Authors: Li L, Wang J, Liu XY, Liu H, Shi HY, Yang XX, Li N, Li YJ, Huang RT, Xue S, Qiu XB, Yang YQ

Abstract
Congenital heart defects (CHDs), a wide variety of developmental abnormalities in the structures of the heart and the great thoracic blood vessels, are the most common form of birth defect in humans worldwide. CHDs are accountable for substantial morbidity and are still the leading cause of birth defect‑related deaths. Recent studies have demonstrated the pivotal roles of genetic defects in the pathogenesis of CHDs, and a great number of genetic mutations have been associated with CHDs. Nevertheless, CHDs are a genetically heterogeneous disorder and the genetic basis underlying CHDs in an overwhelming majority of cases remains unclear. In the present study, the coding exons and flanking introns of the heart and neural crest derivatives expressed transcript 1 (HAND1) gene, which encodes a basic helix‑loop‑helix transcription factor crucial for cardiovascular development, were sequenced in 158 unrelated patients with CHDs, and a de novo heterozygous mutation, p.K132X, was identified in a patient with double outlet right ventricle (DORV), as well as ventricular septal defect. The nonsense mutation, which was predicted to produce a truncated HAND1 protein lacking 84 carboxyl‑terminal amino acids, was absent in 600 control chromosomes. Functional analyses revealed that the HAND1 K132X mutant had no transcriptional activity. Furthermore, the mutation disrupted the synergistic activation between HAND1 and GATA binding protein 4 (GATA4), another cardiac core transcription factor causally linked to CHDs. To the best of our knowledge, this is the first report on the association of HAND1 loss‑of‑function mutation with an enhanced susceptibility to DORV in humans. These findings expand the phenotypic spectrum linked to HAND1 mutations, suggesting potential implications for the development of novelo prophylactic and therapeutic strategies for DORV.

PMID: 28350043 [PubMed - as supplied by publisher]

Infant Motor Skills After a Cardiac Operation: The Need for Developmental Monitoring and Care.

Ann Thorac Surg. 2017 Mar 24;:

Authors: Uzark K, Smith C, Donohue J, Yu S, Romano JC

Abstract
BACKGROUND: Neurodevelopmental dysfunction is increasingly recognized as a common outcome of congenital heart defects and their treatment in infancy. The effects of the intensive care unit (ICU) experience and environment on these infants are unknown and potentially modifiable, but no validated metric is available for objective evaluation of early motor impairments in the ICU/hospital setting. The purpose of this study was to characterize the motor status of hospitalized infants after cardiac operations, including the development and field-testing of the Congenital Heart Assessment of Sensory and Motor Status (CHASMS) metric.
METHODS: CHASMS item generation was based on review of the literature, focused interviews with parents, and expert consensus. A nurse administered CHASMS to 100 infants aged younger than 10 months old undergoing cardiac operations. Preoperative and postoperative CHASMS scores were compared, and associations between CHASMS scores and patient characteristics were examined. Physical therapists assessed neuromotor skills by using the Test of Infant Motor Performance or the Alberta Infant Motor Scales for correlation with CHASMS scores.
RESULTS: CHASMS gross motor scores declined postoperatively in 64% (25 of 39). Lower CHASMS scores, after adjusting for age, were associated with longer duration of mechanical ventilation (p < 0.001) and ICU length of stay (p = 0.001). Gross motor CHASMS scores were significantly correlated with Test of Infant Motor Performance (r = 0.70, p < 0.001) and Alberta Infant Motor Scales scores (r = 0.88, p < 0.001).
CONCLUSIONS: Motor impairments in infants after cardiac operations are common and may be exacerbated by longer intubation and prolonged exposure to the ICU environment. The feasibility, reliability, and validity of CHASMS were supported for the evaluation of motor skills in this at-risk population.

PMID: 28347538 [PubMed - as supplied by publisher]

Closure of coronary artery fistula in childhood: treatment techniques and long-term follow-up.

Clin Res Cardiol. 2017 Mar;106(3):211-218

Authors: Christmann M, Hoop R, Dave H, Quandt D, Knirsch W, Kretschmar O

Abstract
INTRODUCTION: Coronary artery fistula (CAF) is a rare congenital anomalous connection between the coronary arteries (CA) and a cardiac chamber or great vessel. Treatment options of symptomatic CAF consist of transcatheter or surgical closure.
METHODS: Retrospective analysis of all patients with CAF diagnosed between 1993 and 2014 concerning treatment approaches and follow-up after closure.
RESULTS: In a cohort of more than 25000 patients, 194 (<0.01 %) were diagnosed to have CAF. Median age at diagnosis was 6 months (0 day-18 years). Treatment was indicated in ten patients (5.2 %). Six patients (60 %) were treated by catheter interventional approach using Coils (three patients), Amplatzer Vascular Plugs (two patients) and Amplatzer Duct Occluder (one patient). One of these patients showed a significant residual shunt through the fistula 5 days after interventional closure, necessitating surgical removal of the device and closure of CAF. At a median follow-up of 7 (range 2-12) years, the remaining five patients showed successfully closed CAF without causing thrombosis of the CA. Control angiography in three patients showed persistent dilated CA. Surgical closure of CAF was performed in four (40 %) patients; in two as an isolated procedure and in the remaining two as a part of another congenital cardiac corrective procedure.
CONCLUSIONS: CAF in paediatric cardiology patients is a very rare finding. Intervention in childhood is rarely needed; nevertheless, it is known that small fistulas may become relevant in adulthood. Transcatheter closure techniques are effective and are considered the treatment of choice, especially in isolated CAF.

PMID: 27730300 [PubMed - indexed for MEDLINE]

Novel Modifications of a Ventricular Assist Device for Infants and Children.

Ann Thorac Surg. 2016 Jul;102(1):147-53

Authors: Mongé MC, Kulat BT, Eltayeb O, Balasubramanya S, Sarwark AE, Zingle NR, Moss ST, Moga MA, Gossett JG, Pahl E, Costello JM, Backer CL

Abstract
BACKGROUND: A continuous-flow "adult" ventricular assist device (VAD) was modified to support infants and children waiting for heart transplantation.
METHODS: A centrifugal VAD, designed to flow at 1.5 to 8 L/min, was used as a bridge to transplantation in pediatric patients. In smaller children and infants, a modified recirculation shunt permitted lower flow ranges. In hypoxic patients, an oxygenator was spliced into the circuit.
RESULTS: From 2010 to 2015, the VAD was placed in 13 consecutive patients. Age ranged from 0.9 to 16 years (median, 7 years). Body surface area (BSA) ranged from 0.4 to 2.1 m(2) (median, 0.8 m(2)). Ten patients had a BSA less than 1.0 m(2). Four patients were receiving extracorporeal membrane oxygenation (ECMO) before VAD. Three patients had single-ventricle physiology. Five patients had a recirculation shunt and 3 underwent insertion of an oxygenator. Median time on the VAD was 20 days (range, 2-140 days). In patients with a recirculation shunt, mean patient flow was 1.5 L/min (mean flow/BSA, 2.7 L/min/m(2)), with mean total VAD flow of 3.4 L/min. Twelve patients underwent transplantation, and 1 patient underwent VAD explantation. All patients survived and were discharged at a median of 26 days (range, 17-83 days) after transplantation. Three patients experienced major bleeding events. There were 2 cerebrovascular accidents. VAD mortality dropped from 33% (3 of 9) during 2007 to 2010 to 0% (0 of 13) between 2011 and 2015 (p = 0.05). Wait-list mortality dropped from 10% (5 of 52) to 4% (4 of 91) for these periods (p = 0.29).
CONCLUSIONS: The centrifugal VAD successfully supported pediatric patients awaiting heart transplantation. The modified recirculation shunt facilitated the successful support of patients in whom optimal flows were substantially lower than those recommended by the manufacturer. The design allows placement of an in-line oxygenator. Compared with pulsatile devices, use of this VAD was associated with a trend toward decreased mortality associated with VAD use.

PMID: 27240450 [PubMed - indexed for MEDLINE]

Polytetrafluoroethylene Bicuspid Pulmonary Valve Replacement: A 5-Year Experience in 119 Patients With Congenital Heart Disease.

Ann Thorac Surg. 2016 Jul;102(1):163-9

Authors: Lee C, Lee CH, Kwak JG

Abstract
BACKGROUND: Durability of bioprosthetic valves in the pulmonary position is suboptimal. The objective of this study was to evaluate midterm outcomes of hand-sewn polytetrafluoroethylene (PTFE) bicuspid pulmonary valve replacement.
METHODS: A retrospective review of 119 patients who underwent bicuspid pulmonary valve replacement using 0.1-mm PTFE membrane between June 2009 and May 2014 was performed. Reintervention was defined as any surgical or percutaneous catheter procedure on the PTFE pulmonary valve. Structural valve deterioration (SVD) was defined as development of a peak pressure gradient equal to or greater than 50 mm Hg or at least a moderate amount of pulmonary regurgitation on follow-up echocardiography.
RESULTS: The median age at pulmonary valve replacement was 16.9 years (range 5 months to 57.1 years). Fundamental diagnoses were tetralogy of Fallot (n = 71), pulmonary atresia with ventricular septal defect (n = 21), double-outlet right ventricle (n = 13), and others (n = 14). The median valve size was 26 mm (range 12 to 30 mm). Two hospital deaths (1.7%) occurred, from ventricular dysfunction and infective endocarditis. At discharge, no patient showed significant pulmonary regurgitation or stenosis. Follow-up completeness was 94.0%, and the median duration of follow-up was 2.6 years (range 0.1 to 5.2 years). One late death occurred, the result of infective endocarditis involving the PTFE pulmonary valve. Actuarial survival was 97.1% ± 1.6% at 5 years. One patient underwent excision of the PTFE pulmonary valve because of infective endocarditis. Freedom from pulmonary valve reoperation was 99.1% ± 0.9% at 5 years. Ten patients developed SVD during follow-up, and 6 of them underwent an interventional catheter procedure. Freedom from pulmonary valve reintervention and SVD at 5 years was 90.7% ± 3.4% and 86.5% ± 4.0%, respectively.
CONCLUSIONS: Midterm outcomes of hand-sewn PTFE bicuspid pulmonary valve replacement were acceptable. Long-term follow-up of this cohort is mandatory to determine the durability of this valve.

PMID: 27083247 [PubMed - indexed for MEDLINE]

Use of Vertical Expandable Prosthetic Titanium Rib (VEPTR) in the Treatment of Congenital Scoliosis Without Fused Ribs.

J Pediatr Orthop. 2016 Jun;36(4):329-35

Authors: Murphy RF, Moisan A, Kelly DM, Warner WC, Jones TL, Sawyer JR

Abstract
BACKGROUND: Although the vertical expandable prosthetic titanium rib (VEPTR) has been shown to be useful in treating congenital scoliosis (CS) with fused ribs, no studies to date have specifically evaluated the efficacy of VEPTR in the treatment of CS without fused ribs. The purpose of this study was to determine the effectiveness of VEPTR in sagittal/coronal curve correction and spine growth and compare its complication rate to the use of VEPTR in other conditions and to other treatment methods used for CS.
METHODS: A multicenter database was queried for patients with CS without fused ribs treated with VEPTR. Anteroposterior (AP) and lateral radiographs were used to measure parameters at 3 timepoints (preoperative, immediate postoperative, and latest follow-up): coronal Cobb angle, sagittal kyphosis, and thoracic and lumbar spine heights. Clinical data included age, time to follow-up, and complications.
RESULTS: Twenty-five patients (13 females, 12 males) were identified. The average age at implantation was 5.7 years, with an average follow-up of 50 months. Several parameters improved from preoperative to latest follow-up: coronal Cobb angle (69 to 54 degrees, P<0.0001), thoracic spine height (T1-T12) in the AP (13.3 to 15.9 cm, P<0.0001) and lateral (14.8 to 17.4 cm, P=0.0024) planes, and lumbar spine height (L1-S1) in the AP (8.8 to 11.4 cm, P<0.0001) and lateral (9.9 to 11.9 cm, P=0.0002) planes. Kyphosis increased over the study period (36 to 41 degrees, P=0.6). Fifteen patients (60%) had 41 complications (average 2.75; range, 1 to 12). Twenty-eight complications (68%) were device-related, and 13 (32%) were disease-related. The most common complications were infection, wound dehiscence, and device migration. Six complications (15%) altered the course of treatment. Thoracic spine height increased 79% of expected growth.
CONCLUSION: VEPTR is an effective treatment for patients with CS without fused ribs, as evidenced by improved radiographic parameters and increased spinal height, with a complication rate which is high but similar to other methods of treatment.
LEVEL OF EVIDENCE: Level IV-case series.

PMID: 25887832 [PubMed - indexed for MEDLINE]

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Congenital cardiac surgery

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Differentiation of idiopathic spinal cord herniation from dorsal arachnoid webs on MRI and CT myelography.

J Neurosurg Spine. 2017 Mar 24;:1-6

Authors: Schultz R, Steven A, Wessell A, Fischbein N, Sansur CA, Gandhi D, Ibrahimi D, Raghavan P

Abstract
OBJECTIVE Dorsal arachnoid webs (DAWs) and spinal cord herniation (SCH) are uncommon abnormalities affecting the thoracic spinal cord that can result in syringomyelia and significant neurological morbidity if left untreated. Differentiating these 2 entities on the basis of clinical presentation and radiological findings remains challenging but is of vital importance in planning a surgical approach. The authors examined the differences between DAWs and idiopathic SCH on MRI and CT myelography to improve diagnostic confidence prior to surgery. METHODS Review of the picture archiving and communication system (PACS) database between 2005 and 2015 identified 6 patients with DAW and 5 with SCH. Clinical data including demographic information, presenting symptoms and neurological signs, and surgical reports were collected from the electronic medical records. Ten of the 11 patients underwent MRI. CT myelography was performed in 3 patients with DAW and in 1 patient with SCH. Imaging studies were analyzed by 2 board-certified neuroradiologists for the following features: 1) location of the deformity; 2) presence or absence of cord signal abnormality or syringomyelia; 3) visible arachnoid web; 4) presence of a dural defect; 5) nature of dorsal cord indentation (abrupt "scalpel sign" vs "C"-shaped); 6) focal ventral cord kink; 7) presence of the nuclear trail sign (endplate irregularity, sclerosis, and/or disc-space calcification that could suggest a migratory path of a herniated disc); and 8) visualization of a complete plane of CSF ventral to the deformity. RESULTS The scalpel sign was positive in all patients with DAW. The dorsal indentation was C-shaped in 5 of 6 patients with SCH. The ventral subarachnoid space was preserved in all patients with DAW and interrupted in cases of SCH. In no patient was a web or a dural defect identified. CONCLUSIONS DAW and SCH can be reliably distinguished on imaging by scrutinizing the nature of the dorsal indentation and the integrity of the ventral subarachnoid space at the level of the cord deformity.

PMID: 28338452 [PubMed - as supplied by publisher]

Congenital Diaphragmatic hernia - a review.

Matern Health Neonatol Perinatol. 2017;3:6

Authors: Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S

Abstract
Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes. Delivery of infants with CDH is recommended close to term gestation. Immediate management at birth includes bowel decompression, avoidance of mask ventilation and endotracheal tube placement if required. The main focus of management includes gentle ventilation, hemodynamic monitoring and treatment of pulmonary hypertension followed by surgery. Although inhaled nitric oxide is not approved by FDA for the treatment of PPHN induced by CDH, it is commonly used. Extracorporeal membrane oxygenation (ECMO) is typically considered after failure of conventional medical management for infants ≥ 34 weeks' gestation or with weight >2 kg with CDH and no associated major lethal anomalies. Multiple factors such as prematurity, associated abnormalities, severity of PPHN, type of repair and need for ECMO can affect the survival of an infant with CDH. With advances in the management of CDH, the overall survival has improved and has been reported to be 70-90% in non-ECMO infants and up to 50% in infants who undergo ECMO.

PMID: 28331629 [PubMed - in process]

The Role of Conventional and Right-Sided ECG Screening for Subcutaneous ICD in a Tetralogy of Fallot Population.

Pacing Clin Electrophysiol. 2017 Feb;40(2):145-153

Authors: Alonso P, Osca J, Cano O, Pimenta P, Andrés A, Yagüe J, Millet J, Rueda J, Sancho-Tello MJ

Abstract
BACKGROUND: Information regarding suitability for subcutaneous implantable cardioverter-defibrillator (S-ICD) implant in tetralogy of Fallot (ToF) population is scarce and needs to be further explored.
THE AIMS OF OUR STUDY WERE: (1) to determine the proportion of patients with ToF eligible for S-ICD, (2) to identify the optimal sensing vector in ToF patients, (3) to test specifically the eligibility for S-ICD with right-sided screening, and (4) to compare with the proportion of eligible patients in a nonselected ICD population.
METHODS: We recruited 60 consecutive patients with ToF and 40 consecutive nonselected patients. Conventional electrocardiographic screening was performed as usual. Right-sided alternative screening was studied by positioning the left arm and right arm electrodes 1 cm right lateral to the xiphoid midline. The Boston Scientific electrocardiogram (ECG) screening tool was utilized.
RESULTS: We found a higher proportion of patients with right-sided positive screening in comparison with standard screening (77 ± 0.4% vs. 67 ± 0.4%; P < 0.0001) and a trend to higher number of appropriate leads in right-sided screening (1.3 ± 1 vs. 1.1 ± 1 ms; P = 0.07). Patients who failed the screening had a longer QRS duration and longer QT interval. Standard and right-sided screening showed a higher percent of positive patients in the control group compared to ToF patients (P < 0.001).
CONCLUSION: Right-sided screening was associated with a significant 10% increase in S-ICD eligibility in ToF patients. When comparing with an acquired cardiomyopathies group, ToF showed a lower eligibility for S-ICD. The most appropriate ECG vector was the alternate vector in contrast to what is observed in the general population.

PMID: 28070890 [PubMed - indexed for MEDLINE]

A Rare Congenital Pulmonary Anomaly of a Young Adult: Pseudosequestration.

Ann Thorac Surg. 2016 Aug;102(2):e163

Authors: Özdil A, Akçam Tİ, Çağırıcı U, Savaş R

PMID: 27449457 [PubMed - indexed for MEDLINE]

The Impact of Dominant Ventricle Morphology on Palliation Outcomes of Single Ventricle Anomalies.

Ann Thorac Surg. 2016 Aug;102(2):593-601

Authors: Alsoufi B, Gillespie S, Kim D, Shashidharan S, Kanter K, Maher K, Kogon B

Abstract
BACKGROUND: Differences in right ventricle and tricuspid valve structure compared with left ventricle and mitral valve structure make them less equipped to support the systemic circulation long term, with subsequent systemic right ventricle failure. We examined the effect of dominant ventricle morphology on single ventricle palliation outcomes.
METHODS: We grouped 530 neonates who underwent first-stage palliation into two groups based on dominant ventricle morphology: right dominant ventricle (RV group; n = 302, 57%) and left dominant ventricle or functional single ventricle with two well-formed ventricles (LV group; n = 228, 43%). Comparisons of hospital outcomes, interstage mortality, progression to subsequent palliation stages, and late survival was performed, and factors affecting outcomes were examined.
RESULTS: After first-stage palliation, the RV group and LV group, respectively, had comparable extracorporeal membrane oxygenation requirements (12% versus 11%, p = 0.648), unplanned reoperation (12% versus 13%, p = 0.586), and hospital death (16% versus 13%, p = 0.437). Among hospital survivors, interstage mortality (11% versus 9%, p = 0.509) and progression to Glenn operation (89% versus 84%, p = 0.182) were comparable; however, death after Glenn was higher in the RV group (10%, versus LV group 4%, p = 0.020) with a trend for lower 8-year survival (66% versus 73%, p = 0.081). On multivariable analysis, dominant RV was not associated with mortality (hazard ratio 0.75, 95% confidence interval: 0.6 to 1.0, p = 0.081), whereas factors such as genetic syndromes, weight 2.5 kg or less, underlying cardiac anomaly, and first-stage palliation type affected survival.
CONCLUSIONS: At midterm follow-up, underlying cardiac anomaly and patient characteristics affect single ventricle palliation outcomes more than dominant ventricular morphology. As right ventricle and associated tricuspid valve failure might occur at late stages, the impact of dominant ventricular morphology on long-term outcomes requires further assessment.

PMID: 27345094 [PubMed - indexed for MEDLINE]

Current Outcomes of Surgical Management of Aortopulmonary Window and Associated Cardiac Lesions.

Ann Thorac Surg. 2016 Aug;102(2):608-14

Authors: Alsoufi B, Schlosser B, McCracken C, Kogon B, Kanter K, Border W, Sachdeva R

Abstract
BACKGROUND: Aortopulmonary window (APW) is a rare congenital defect that is often associated with other cardiac lesions. We analyzed our operative strategy to determine whether this had any relationship with outcomes.
METHODS: Early and late outcomes of 40 children who underwent APW repair at our institution during a 20-year period (1994 to 2013) were analyzed.
RESULTS: Median age at time of the operation was 22 days (interquartile range, 9 to 63 days), and median weight was 3.2 kg (interquartile range, 2.5 to 3.8 kg). Eleven patients (28%) were born prematurely at or before 36 weeks' gestation, and 10 (25%) had genetic/extracardiac malformations. Mean APW size was 0.84 ± 0.28 cm, and by the Mori classification was type I in 17 patients (43%), type II in 18 (45%), and type III in 5 (13%). Twenty-five patients (63%) had simple APW, with no associated cardiac lesions other than atrial septal defect or patent ductus arteriosus, whereas 15 (38%) had complex APW with one or more associated lesions, including interrupted aortic arch (n = 6), ventricular septal defect (n = 6), or other (n = 5). There were no hospital or late deaths. Four patients required cardiac reoperations, 3 of whom had interrupted aortic arch. The 10-year freedom from cardiac reoperation was 100% for simple APW vs 73% for complex APW (p = 0.008), with 75% of reoperations related to aortic obstruction. Age, weight, prematurity, extracardiac anomalies, APW size and type, and APW repair technique were not associated with reoperation risk.
CONCLUSIONS: Current outcomes of early repair of APW are excellent, including infants with complex associated cardiac lesions. Compared with historic results, contemporary outcomes are favorable, supporting early and complete repair of APW and associated lesions. Cardiac reoperation can be required in complex APW, mainly with concomitant arch repair, and is usually related to aortic obstruction.

PMID: 27207392 [PubMed - indexed for MEDLINE]

New Technique for Surgical Epicardial Implantation of a Cardioverter-Defibrillator in Children and Adults With Congenital Heart Disease.

Ann Thorac Surg. 2016 Aug;102(2):615-9

Authors: Pospiech T, Roubertie F, Jalal Z, Bordachar P, Thambo JB

Abstract
BACKGROUND: We assessed a surgical technique for implanting a cardioverter-defibrillator. The indications for cardioverter-defibrillator implantation in pediatric patients and adults with congenital heart disease are relatively specific and require multidisciplinary discussion regarding implantation modalities. We coupled the positioning of two coils sutured to the pericardium with an implantable cardioverter-defibrillator device inserted within a supradiaphragmatic pocket in a population of children and adults with congenital heart disease.
METHODS: Thirteen consecutive patients, either children or adults with congenital heart disease, underwent the implantation of a single-chamber implantable cardioverter-defibrillator in our center. All patients were systematically followed at 3, 6, and 12 months after implantation.
RESULTS: Patients were mainly male (n = 9, 69%), and mean age was 21 ± 12 years. Median follow-up was 13 months. All patients underwent surgery without acute complication. One patient needed a second surgery because of defibrillation coils fracture. Neither infectious complication nor inappropriate shock was noted. There were two appropriate shocks in 1 patient.
CONCLUSIONS: This new technique for surgical epicardial implantation of a cardioverter-defibrillator in children and adults with congenital heart disease is safe and feasible. These results should be confirmed by prospective studies with long-term follow-up.

PMID: 27207391 [PubMed - indexed for MEDLINE]

Long-Term Survival and Freedom From Reoperation After Placement of a Pulmonary Xenograft Valved Conduit.

Ann Thorac Surg. 2016 Aug;102(2):602-7

Authors: Alfieris GM, Swartz MF, Lehoux J, Bove EL

Abstract
BACKGROUND: The optimal choice for pulmonary valve replacement (PVR) remains controversial. This study hypothesized that xenografts used for PVR would result in prolonged long-term survival and freedom from reoperation.
METHODS: Children and adults with congenital heart disease requiring PVR using a xenograft from 1980 to 1985 were reviewed. In all cases, the xenograft valve was either sewn or manufactured into a Dacron conduit, and the conduit was sewn to the pulmonary artery bifurcation. Clinical data were analyzed, and survival and freedom from reoperation were determined using Kaplan-Meier analysis.
RESULTS: Twenty-four patients received a xenograft for PVR at 14.6 ± 5.6 years. Conduit size ranged from 21 to 27 mm. Most patients received a Carpentier-Edwards valved conduit (n = 17), followed by a Hancock valved conduit (n = 5) and an Ionescu-Shiley valve sewn into a Dacron graft (n = 2). No perioperative deaths occurred. Reoperation was required mainly for pulmonary stenosis (72.7%), followed by pulmonary insufficiency (18.2%), or both stenosis and insufficiency (9%). Freedom from reoperation was 90%, 56%, 43%, and 14% at 10, 20, 25, and 30 years, respectively. At most recent follow-up the was only death, which was related to severe biventricular failure 25 years after conduit implant.
CONCLUSIONS: PVR using a xenograft valved conduit results in prolonged freedom from reoperation and excellent long-term survival. These data, which provide long-term follow-up information on xenograft valves after PVR.

PMID: 27154149 [PubMed - indexed for MEDLINE]

Seminal Postoperative Complications and Mode of Death After Pediatric Cardiac Surgical Procedures.

Ann Thorac Surg. 2016 Aug;102(2):628-35

Authors: Gaies M, Pasquali SK, Donohue JE, Dimick JB, Limbach S, Burnham N, Ravishankar C, Ohye RG, Gaynor JW, Mascio CE

Abstract
BACKGROUND: Understanding the seminal complications leading to death after pediatric cardiac surgical procedures may provide opportunities to reduce mortality. This study analyzed all deaths at two pediatric cardiac surgical programs and developed a method to identify the seminal complications and modes of death.
METHODS: Trained nurses abstracted all cases of in-hospital mortality meeting inclusion criteria from each site over 5 years (2008 to 2012). Complication definitions were consistent with those of a multicenter clinical registry. An adjudication committee assigned a seminal complication in each case (the complication initiating the cascade of events leading to death). Seminal complications were grouped into categories to designate "mode of death." The epidemiology of seminal complications and of mode of death was described.
RESULTS: In 191 subjects, low cardiac output syndrome (71% of all subjects), cardiac arrest (52%), and arrhythmia (48%) were the most common complications. The committee assigned low cardiac output syndrome (30%), failure to separate from bypass (16%), and cardiac arrest (12%) most frequently as seminal complications. Seminal complications occurred a median 2 hours (interquartile range [IQR], 0 to 35 hours) postoperatively. Patients experienced a median of seven (IQR, 3 to 12) additional complications before death at a median of 15 days (IQR, 4 to 46). Systemic circulatory failure was the most common mode of death (51%), followed by inadequate pulmonary blood flow (13%) and cardiac arrest (12%).
CONCLUSIONS: Seminal complications occurred early postoperatively, and systemic circulatory failure was the most common mode of death. Our classification system is likely scalable for subsequent multicenter analysis to understand cause-specific mortality variation across hospitals and to drive quality improvement.

PMID: 27154145 [PubMed - indexed for MEDLINE]

Standardization of reporting would help to define best treatment for pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries.

Eur J Cardiothorac Surg. 2016 Jul;50(1):167-8

Authors: d'Udekem Y

PMID: 27044899 [PubMed - indexed for MEDLINE]

Factors affecting death and progression towards next stage following modified Blalock-Taussig shunt in neonates.

Eur J Cardiothorac Surg. 2016 Jul;50(1):169-77

Authors: Alsoufi B, Gillespie S, Mori M, Clabby M, Kanter K, Kogon B

Abstract
OBJECTIVES: The modified Blalock-Taussig shunt (BTS) is utilized to palliate neonates born with restrictive pulmonary blood flow including those with single ventricle (SV) or biventricular (BV) cardiac anomalies. We aim in the current study to report palliation outcomes of neonates with BTS and to examine factors affecting death and progression to the subsequent stage of palliation or repair.
METHODS: Between 2002 and 2012, 341 patients underwent BTS including 175 with SV and 166 with BV anomalies. Competing risk analysis modelled events after BTS (death or transplantation, transition to Glenn shunt or biventricular repair) and examined risk factors affecting outcomes.
RESULTS: SV patients had a higher incidence of extracorporeal membrane oxygenation (ECMO) support requirement (12 vs 4%, P = 0.004) and unplanned cardiac reoperation (14 vs 7%, P = 0.051) than their BV counterparts. Additionally, hospital mortality was higher in SV than in BV patients (15 vs 3%, P < 0.001). In SV patients, competing risk analysis showed that, 2 years following BTS, 27% of patients had died or received transplantation and 73% had undergone the Glenn shunt. On multivariable analysis, factors associated with time until death or transplantation prior to Glenn were cardiopulmonary bypass [hazard ratio (HR) 3.6 (2.0-6.4), P < 0.001], unplanned cardiac reoperation [HR 2.4 (1.3-4.6), P = 0.007], pulmonary atresia [HR 2.0 (1.1-3.7), P = 0.026] and the shunt size/weight ratio [HR 1.3 (1.1-1.4) per 0.1 increase, P = 0.001]. In BV patients, competing risk analysis showed that, 2 years following BTS, 13% of patients had died or received transplantation, 85% had undergone biventricular repair and 2% were alive without biventricular repair. On multivariable analysis, factors associated with time until death or transplantation prior to biventricular repair were genetic syndromes and extracardiac malformations [HR 6.1 (2.0-18.2), P = 0.001], weight ≤2.5 kg [HR 5.6 (2.0-16.0), P = 0.001] and male gender [HR 3.4 (1.1-11.0), P = 0.041].
CONCLUSIONS: Palliation with BTS continues to be associated with significant operative morbidity and mortality. In addition to hospital death, there is an important interstage attrition risk prior to subsequent palliation or biventricular repair. Inherent patient characteristics (i.e. genetic syndromes and low weight) and anatomical details (i.e. SV, pulmonary atresia and concomitant cardiac anomalies) are associated with worse survival.

PMID: 26912652 [PubMed - indexed for MEDLINE]

An uncommon cause of tricuspid regurgitation: three-dimensional echocardiographic incremental value, surgical and genetic insights.

Eur J Cardiothorac Surg. 2016 Jul;50(1):180-2

Authors: Theron A, Pinard A, Riberi A, Zaffran S

Abstract
Congenital tricuspid valve disease is a rare defect that includes regurgitation, stenosis and Ebstein's anomaly. We report a case of severe tricuspid regurgitation associated with functional mitral regurgitation in a 47-year-old man with congestive heart failure. Transthoracic echocardiography (TTE) ruled out any Ebstein's anomaly. Three-dimensional TTE revealed a 'tricuspid hole' into the anterior leaflet that was only attached to the tricuspid annulus next to both anteroseptal and anteroposterior commissures. There was no sign of leaflet tear or perforation. The surgical repair of the tricuspid and mitral valves was performed with an optimal result. No sign of endocarditis or rheumatic disease was observed during the intervention. Sequence analysis of GATA4, HEY2 and ZFPM2 genes was performed, but no causative mutation was identified.

PMID: 26670804 [PubMed - indexed for MEDLINE]

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Congenital cardiac surgery

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