Aortic aneurysms after correction of aortic coarctation: a systematic review.

Despite advanced techniques for surgical or percutaneous therapy coarctation of the aorta continues to carry a high risk of aneurysmal formation. Mortality of these aneurysms ranges between <1 and >90%, reflecting remarkable differences in surgical strategies and the follow-up management of coarctation. We review the frequency, anatomical types, risk factors and mechanisms of aortic aneurysm forming late after surgical or percutaneous therapy of aortic coarctation. We emphasize that aneurysms do not form exclusively at the site of previous intervention, but also at remote locations such as the ascending aorta. Moreover, aneurysm formation may only in part be attributed to a specific technique of coarctation therapy, and we emphasize the role of a bicuspid aortic valve and inherent weakness of the aortic wall as significant risk factors for aneurysm after aortic coarctation. We report the presenting symptoms, follow-up protocols, and imaging criteria for local and proximal aneurysms. Finally, we discuss criteria for prophylactic intervention at the site of such aneurysms, and present therapeutic options for different types of aneurysms. With this systematic review, we wish to provide data for establishing more uniform strategies for preventing, diagnosing and treating aneurysms associated with aortic coarctation.

Marfan syndrome and the evolving spectrum of heritable thoracic aortic disease: do we need genetics for clinical decisions?

Marfan syndrome (MFS) is a disorder of the connective tissue that is inherited in an autosomal dominant fashion and that is classically caused by mutations in the gene coding for fibrillin-1, FBN1. The high mortality of untreated MFS results almost exclusively from aortic complications such as aortic dissection and rupture. However, more than half of patients with Marfan-like features do not have MFS, but have other diseases including inherited aortic aneurysms and dissections (TAAD). We elucidate the increasing spectrum of syndromes associated with Marfan-like features and discuss the clinical implications of these diseases. We performed a systematic review to tabulate all known inherited diseases and syndromes carrying a risk for thoracic aortic disease. We discuss evidence that different syndromes with different causative genes and mutations have different prognoses and profiles of cardiovascular manifestations. We conclude that future decisions for optimized management of patients with inherited TAAD require a comprehensive clinical and genetic work-up.

Clinically relevant tracheomalacia after repair of esophageal atresia: the role of minimal intra-operative dissection and timing for aortopexy.

BACKGROUND: Tracheomalacia is associated with esophageal atresia (EA), but may go unnoticed from external splinting forces. Intra-operative dissection with fistula division releases external splinting, revealing tracheomalacia only post-operatively. Analysis of surgical technique may disclose an iatrogenic etiology. METHODS: From 1995 - 2004, 44 neonates underwent surgery for EA. All patients underwent pre-, intra- and postoperative bronchoscopy. Operative and bronchoscopic notes were studied for malacia, and extensive dissection of the esophagus and fistula from the trachea. RESULTS: Surgical mortality was 6.8 %. Pre-operative tracheomalacia was diagnosed in 3 patients, who eventually fared well. In 17 other patients, the pre-operative bronchoscopy was negative, but airway obstruction developed post-operatively. Tracheomalacia was documented at the site of the former fistula and surgical release maneuvers. Aortopexy was required in 5 instances, whereas 12 others with malacia were managed conservatively. CONCLUSIONS: Mediastinal connective tissue and the fistula may splint open the marginally diseased airway in patients with EA, the lack of which may disclose previously unknown tracheomalacia after repair. When aggressive release maneuvers have been required, early aortopexy may be preferred to ventilator dependency.

Challenges encountered during closure of atrial septal defects.

Since the introduction of transcatheter interventional occlusion of atrial septal defects (ASDs) by King and coworkers, the device closure has become the standard treatment of selected atrial defects. Although it has become routine for standard clinical practice, increasing knowledge of intracardiac anatomy, modification of implantation techniques, and improvements in device designs are making this treatment modality increasingly applicable to a wider range of selected patients. This report summarizes the current trends in patient selection, focusing on the anatomical limits of applicability for ASD device closure, and discusses the future possibilities. Furthermore, typical complications are described with regard to the rare erosion of the atrial roof or the aortic root. Current practice is to oversize the device if the anterior rim toward the aorta tends to be diminutive, achieving a secure position of the device around the aortic root. This and the shape memory of the device may play a major role in this serious complication and should be reconsidered. Although most interventional occlusions of interatrial defects with a device have become clinically routine, some situations remain highly challenging.

Topical vascular endothelial growth factor in rabbit tracheal surgery: comparative effect on healing using various reconstruction materials and intraluminal stents.

OBJECTIVES: The effect of topical vascular endothelial growth factor (VEGF) on post-surgical tracheal healing using various reconstruction materials was studied, with particular regard to prevention of granulation tissue or fibrosis. METHODS: Twenty-four New Zealand White rabbits underwent survival surgery using autograft patches (n=6), xenopericardium patches (n=6), intraluminal Palmaz wire stents (n=6), and controls (n=6). Autograft and pericardial half-patches were soaked in topical VEGF (5 microg/ml over 30 min) and saline before reimplantation. Stents and controls received circumferential injections of VEGF and saline in the tracheal wall. At 1-4 months postoperatively, specimens of sacrificed animals were stained with anti-VEGF antibody, followed by morphological and immunohistochemical examination. RESULTS: Rabbits with autografts and controls fared well until planned sacrifice. After xenopericardium repair, obstructive intraluminal granulation tissue led to early sacrifice in three rabbits. Stent insertion led to earlier death from airway obstruction in all six rabbits. Topical VEGF reduced granulation tissue after pericardial repair and fibrosis in all repairs except in stents. Remarkably, VEGF-pretreated half-patches and saline half-patches stained similarly high for VEGF, suggesting also local production of VEGF, probably in plasmacells, and in submucosal glands. CONCLUSIONS: Autograft repair induces the least granulation tissue and fibrosis, and the best healing pattern. Stents rapidly induced critical airway obstruction, unhindered by VEGF, leading to premature death. Tracheal pretreatment with topical VEGF reduces postoperative fibrosis after autograft and pericardial patch repairs, and reduces granulation tissue after xenopericardium repair. In time, VEGF is probably locally produced, although its potential role in tracheal healing remains to be established.

Systemic plasma vascular endothelial growth factor levels as a marker for increased angiogenesis during the single ventricle surgical pathway.

Cyanosis and the cavopulmonary anastomosis (CPA) are associated with pulmonary arterio-venous malformations (PAVMs) in single ventricle physiology. Vascular endothelial growth factor (VEGF) may be a marker of abnormal angiogenesis in this setting. Plasma VEGF levels were measured in 14 patients undergoing the surgical pathway leading to total cavopulmonary connection (TCPC). Venous blood samples were taken before and then months after CPA (n=6), and immediately before TCPC and 1 month thereafter (n=9). Corresponding arterial saturations were correlated with VEGF levels at each time frame. In six patients, pre-CPA plasma VEGF levels rose from a mean of 24.4-112.4 pg/ml (p<0.03) just prior to completion of TCPC. In nine patients, VEGF levels diminished from 115.7 to 48.9 pg/ml (p<0.05) after TCPC. VEGF levels were disproportionately elevated to arterial saturations most notably after CPA (r2=0.002), suggesting an additional angiogenic stimulus besides cyanosis. Plasma VEGF levels fluctuate during the single ventricle surgical pathway, with maximal levels after CPA, and regression after completion of TCPC. High VEGF levels are disproportionate to hypoxia after CPA, potentially incriminating the absence of hepatic flow to the lungs as an abnormal angiogenic stimulus. Measuring VEGF in venous blood may serve as a biochemical marker of angiogenesis after CPA.

The double-switch procedure for atrioventricular and ventriculoarterial discordance.

BACKGROUND: Late ventricular failure remains a major concern in patients with congenitally corrected transposition of the great arteries (ccTGA). A new treatment for this condition is the double-switch procedure. METHODS: Three consecutive children with atrioventricular and ventriculoarterial discordance (congenitally corrected transposition of the great arteries) and associated ventricular septal defect underwent pulmonary artery banding in infancy, followed by a double-switch procedure and closure of the ventricular septal defect at a median age of 5.8 years (range 4.5 to 6 years). RESULTS: There were no major procedure-related complications and the median duration of hospital stay was 13 days. One patient required stent implantation in the superior vena cava five months after surgery to relieve a persistent caval stenosis associated with recurrent pleural and pericardial effusions. Apart from this, no other complications have occurred over a median follow-up of five months. CONCLUSION: The double-switch procedure offers the potential advantage of restoring the morphological left ventricle to systemic ventricle. Longer-term follow-up of this procedure is warranted.

ECG determinants in adult patients with chronic right ventricular pressure overload caused by congenital heart disease: relation with plasma neurohormones and MRI parameters.

OBJECTIVE: To examine retrospectively the changes in ECG parameters over time and their correlation with other quantitative right ventricular (RV) function parameters in patients with chronic RV pressure overload caused by congenital heart disease. METHODS: 48 patients with chronic RV pressure overload caused by the following congenital heart diseases were studied: nine with congenitally corrected transposition of the great arteries (TGA), 12 with surgically corrected TGA, and 27 with a subpulmonary pressure overloaded RV. QRS duration and dispersion were measured manually from standard ECG recorded twice within five years. RV end diastolic volume (EDV) and RV mass were determined by magnetic resonance imaging. Brain natriuretic peptide (BNP) plasma concentrations were measured. RESULTS: QRS duration and QRS dispersion increased in all patient groups during the follow up period. QRS duration increased significantly in the congenitally corrected TGA (p = 0.04) and the subpulmonary pressure overloaded RV groups (p = 0.01). QRS dispersion increased significantly in patients with surgically corrected TGA (p = 0.03) and in the subpulmonary pressure overloaded RV group (p = 0.02). A significant correlation was found between QRS duration and RVEDV (r = 0.71, p < 0.0001). RV mass was significantly correlated with QRS duration in patients with tetralogy of Fallot (r = 0.67, p = 0.01). Mean (SD) plasma brain natriuretic peptide concentrations (6.6 (5.4) pmol/l) were increased compared with normal reference values but no correlation was found with ECG parameters or RV systolic pressure. No malignant arrhythmia or sudden death occurred. CONCLUSIONS: ECG parameters worsened gradually in asymptomatic or minimally symptomatic patients with chronic RV pressure overload, regardless of the nature of their congenital heart disease. In all patients, a significant positive correlation was found between QRS duration and RVEDV. In patients with tetralogy of Fallot there was also a correlation between QRS duration and RV mass.

Impact of junctional ectopic tachycardia on postoperative morbidity following repair of congenital heart defects.

OBJECTIVE: To determine the incidence of postoperative junctional ectopic tachycardia (JET), we reviewed 343 consecutive patients undergoing surgery between 1997 and 1999. The impact of this arrhythmia on in-hospital morbidity and our protocol for treatment were assessed. METHODS: We reviewed the postoperative course of patients undergoing surgery for ventricular septal defect (VSD; n=161), tetralogy of Fallot (TOF; n=114), atrioventricular septal defect (AVSD; n=58) and common arterial trunk (n=10). All patients with JET received treatment, in a stepwise manner, beginning with surface cooling, continuous intravenous amiodarone, and/or atrial pacing if the haemodynamics proved unstable. A linear regression model assessed the effect of these treatments upon hours of mechanical ventilation, and stay on the cardiac intensive care unit (CICU). RESULTS: Overall mortality was 2.9% (n=10), with three of these patients having JET and TOF. JET occurred in 37 patients (10.8%), most frequently after TOF repair (21.9%), followed by AVSD (10.3%), VSD (3.7%), and with no occurrence after repair of common arterial trunk. Mean ventilation time increased from 83 to 187 h amongst patients without and with JET patients (P<0.0001). Accordingly, CICU stay increased from 107 to 210 h when JET occurred (P<0.0001). Surface cooling was associated with a prolongation of ventilation and CICU stay, by 74 and 81 h, respectively (P<0.02; P<0.02). Amiodarone prolonged ventilation and CICU stay, respectively, by 274 and 275 h (P<0.05; P<0.06). CONCLUSIONS: Postoperative JET adds considerably to morbidity after congenital cardiac surgery, and is particularly frequent after TOF repair. Aggressive treatment with cooling and/or amiodarone is mandatory, but correlates with increased mechanical ventilation time and CICU stay. Better understanding of the mechanism underlying JET is required to achieve prevention, faster arrhythmic conversion, and reduction of associated in-hospital morbidity.

Healing of a free tracheal autograft is enhanced by topical vascular endothelial growth factor in an experimental rabbit model.

OBJECTIVE: In 1996, we introduced the free tracheal autograft technique for repair of congenital tracheal stenosis from complete tracheal rings in infants and children. Sources of possible concern with this procedure include the potential for autograft ischemia, patch dehiscence, and recurrent stenosis. Vascular endothelial growth factor is a potent angiogenic inducer (particularly in the setting of ischemia, hypoxia, or both) and is postulated to promote tissue healing. The purpose of this study was to test the hypothesis that pretreatment of tracheal autografts with topical vascular endothelial growth factor would enhance tracheal healing. METHODS: In a rabbit model of tracheal reconstruction (n = 32), an elliptically shaped portion of the anterior tracheal wall was excised. The excised portion of trachea was one third of the tracheal circumference and 2 cm in length (6 tracheal rings). This portion of trachea (the autograft) was soaked in either vascular endothelial growth factor (5 microg/mL, n = 16) or normal saline solution (n = 16) for 15 minutes before being reimplanted in the resultant tracheal opening. Animals were killed and autografts were examined at 2 weeks, 1 month, and 2 months postoperatively for gross and microscopic characteristics. RESULTS: By 2 weeks, and progressing through 1 and 2 months, autografts treated with vascular endothelial growth factor, as compared with control autografts, had reduced luminal stenosis, submucosal fibrosis, and inflammatory infiltrate (P <.05). The autografts tended to become malaligned in control animals, whereas the tracheal architecture was preserved in rabbits treated with vascular endothelial growth factor. Microvascular vessel density was significantly greater in all vascular endothelial growth factor groups (P <.05) at all time intervals. CONCLUSIONS: Topical treatment of free tracheal autografts with vascular endothelial growth factor in a rabbit tracheal reconstruction model enhanced healing, as evidenced by accelerated autograft revascularization, reduced submucosal fibrosis and inflammation, and preservation of the normal tracheal architecture. Topical vascular endothelial growth factor may improve future results of tracheal reconstruction.

Congenital Heart Surgery Nomenclature and Database Project: anomalies of the coronary arteries.

The extant nomenclature for coronary artery anomalies is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The seven major categories of coronary artery anomalies are: anomalous pulmonary artery origins of the coronaries, anomalous aortic origins of the coronaries, congenital atresia of the left main coronary artery, coronary artery fistulas, coronary artery bridging, coronary aneurysms, and coronary stenosis. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail, which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented, which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

A comparison of steroid-eluting epicardial versus transvenous pacing leads in children.

OBJECTIVE: To evaluate the acute and chronic performance of steroid-eluting (SE) epicardial (EPI) pacing leads as compared to SE transvenous (TV) pacing leads in children. METHODS: From 1989 through 1997, 55 children with congenital heart disease received a total of 85 SE pacing leads, of which 38 were EPI and 47 TV. The mean age of children receiving EPI leads was younger than those receiving TV leads (7.7 months vs 15.1 years, p = 0.001), and they had shorter follow-up (17.2 months vs 36.2 months, p < 0.001). All leads were evaluated for acute and chronic sensing and capture thresholds, and impedance. RESULTS: Acute and in particular chronic atrial and ventricular sensing and capture thresholds in SE EPI and TV leads were essentially equivalent. [table: see text]. The chronic impedance of TV leads (atrial 525 ohms, ventricular 520 ohms) was consistently higher than EPI leads (atrial 404 ohms, ventricular 386 ohms). CONCLUSION: At intermediate follow-up, SE EPI leads are functionally equivalent to SE TV leads. We recommend the use of SE EPI leads as long as practically feasible prior to using the TV approach in children who will require a life-time of pacing.

Risk factors for recoarctation and results of reoperation: a 40-year review.

BACKGROUND: We analyzed a single institution 40-year experience with children that had coarctation repair to define risk factors for recoarctation and to evaluate the results of reoperation for recoarctation. MATERIAL AND METHODS: Between 1957 and 1998, 271 patients had primary surgical repair of coarctation of the aorta. Techniques for primary repair included Gore-tex patch aortoplasty (PATCH; n = 118), resection with extended end-to-end anastomosis (RXEEA; n = 69), subclavian flap aortoplasty (SFA; n = 61), resection with simple end-to-end anastomosis (ETE; n = 18), resection and interposition graft (n = 4), and extra-anatomic graft (n = 1). Techniques for recoarctation repair included PATCH, interposition graft, and extra-anatomic graft. RESULTS: Median age at initial repair was 156 days. Major associated cardiac anomalies were present in 96 patients (35%). A hypoplastic aortic arch was present in 37 patients (14%). There were three early deaths (1%) and six late deaths (2%). One patient had paraplegia (0.4%). Recoarctation occurred in 29 patients (11%) and was most frequent with ETE and SFA repairs (33% and 20%, respectively). Multiple logistic regression analysis revealed ETE repair (p = 0.0002), SFA repair (p = 0.049), and aortic arch hypoplasia (p = 0.0001) to be risk factors for recoarctation. Using PATCH as the covariate, the odds ratio to develop recoarctation was 3.5 for SFA, 17.2 for ETE, and 15.2 for hypoplastic aortic arch. There was no mortality or paraplegia after recoarctation repair (n = 23). Six patients had seven balloon angioplasties; two of these patients later required reoperation. Three patients required a second reoperation for persistent coarctation. CONCLUSIONS: In our 40-year review, simple end-to-end anastomosis and subclavian flap repair had the highest incidence of recoarctation, especially when associated with aortic arch hypoplasia. We recommend resection with extended end-to-end anastomosis for repair of neonates and infants and Gore-tex patch aortoplasty for children over the age of 1 year. Surgical repair of recurrent coarctation is safe, effective, and has a low incidence of persistent coarctation.

Management of congenital tracheal stenosis: a multidisciplinary approach.

Congenital tracheal stenosis is a rare but underdiagnosed anomaly which can present as life-threatening respiratory insufficiency in neonates and infants. Initial control of the airway is mandatory. Surgical correction is the mainstay of therapy and is achieved with low mortality. The type and extent of repair depends largely on the length of stenosis. Cardiac anomalies are frequently associated and may be addressed at the time of tracheal surgery. Despite initial satisfactory results, post-operative morbidity due to persistent granulation tissue is substantial. It is through a multidisciplinary approach and close follow-up of the repaired airway that these demanding patients are best cared for. The long-term quality of life remains uncertain.

Right aortic arch, right ligamentum, absent left pulmonary artery: a rare vascular ring.

A 5-month-old infant presented with respiratory failure secondary to severe right bronchial compression. Diagnostic imaging revealed a right aortic arch and absent left pulmonary artery. Surgical relief was obtained via median sternotomy by dividing a right ligamentum and pexing the enlarged right pulmonary artery to the ascending aorta.

Complete repair of Tetralogy of Fallot with absent pulmonary valve including the role of airway stenting.

Tetralogy of Fallot (TOF) with absent pulmonary valve (APV) represents an extreme form of tetralogy where pulmonary insufficiency and mild annular stenosis often results in massive pulmonary arterial (PA) dilatation. The aneurysmal left and right PAs often compress the adjacent trachea and bronchi, leading to airway obstruction and respiratory failure in infancy. Between 1991 and 1997, 11 patients underwent a single stage repair of TOF and APV using a valved (10 patients) or nonvalved (1 patient) homograft conduit and PA reduction arterioplasty. There was one (1/11 [9.1%) perioperative and one (9.1%) late death. Both deaths were related to airway complications. Morbidity associated with postoperative respiratory complications and ventilator-dependency due to underlying tracheobronchomalacia is an important problem. Intermediate follow-up shows a high incidence of reintervention for conduit stenosis and/or insufficiency and tracheobronchial compression. These infants also required multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Stenting of the right and left main bronchi with balloon expandable metallic stents is a new experimental therapy that has been useful in two recent patients with respiratory failure despite satisfactory intracardiac repair. It may provide an attractive alternative therapy to prolonged mechanical ventilation with positive end expiratory pressure in patients with severe tracheobronchomalacia. Complete repair with a valved homograft conduit and reduction pulmonary arterioplasty in infancy at the time of diagnosis is the procedure of choice for infants with TOF with APV. With this approach the patient outcome is essentially determined by their airway status and airway management.