Comparison of antioxidant reserve capacity of children with acyanotic & cyanotic congenital heart disease.

BACKGROUND & OBJECTIVES: Oxidative stress can cause many diseases and increases the risk of post-operative complications in children with congenital heart disease. For these reasons, this study was aimed to investigate the differences between cyanotic and acyanotic paediatric patients who underwent heart surgery with markers of oxidative stress. METHODS: Eighty five patients were included in the study. The samples taken before the surgery and within the first 24 h after the surgery were evaluated for haemoglobin (Hb), leukocytes, uric acid, glutathione (GSH), malondialdehyde and total antioxidant capacity. Cyanotic, acyanotic, hyperoxygenated, normo-oxygenated, cardiac surgery with or without cardiopulmonary bypass (CPB) comparisons were made. RESULTS: Positive correlation was found between age and pre-operative total antioxidant status values. Cyanotic and acyanotic patients did not have different antioxidant reserve capacities preoperatively. Although pre-operative thiobarbituric acid reactive substances (TBARS) levels were significantly lower in cyanotic patients, post-operative levels were higher. TBARS levels increased and GSH levels reduced postoperatively. The level of oxygenation did not cause a significant difference on markers of oxidative stress. The duration of CPB did not have negative effects on oxidative stress. INTERPRETATION & CONCLUSIONS: Cyanotic and younger patients were found to be more vulnerable to oxidative stress. The increased levels of TBARS and the decreased levels of GSH could be the indicators of oxidative damage depending on many factors such as surgery, CPB, ischaemia/reperfusion, inflammation, iron overload and oxygenation. The level of oxygenation does not cause a noticeable difference in oxidative stress. CPB causes oxidative stress, but if it is conducted appropriately, the duration of CPB does not cause a significant negative impact on oxidative stress.

Predictors for Progression of Tricuspid Insufficiency Following Left-Sided Valvular Surgery: A Retrospective Cohort Study.

BACKGROUND: The operative indications for severe tricuspid insufficiency in patients undergoing left-sided valvular surgery are well defined; however, for mild and moderate insufficiency, the findings are still controversial. In this study, we aimed to document the prognostic parameters for progression of tricuspid insufficiency in patients undergoing left-sided valvular surgery. METHODS: 135 patients undergoing mitral valve ± coronary bypass surgery were retrospectively examined. Patients with simultaneous tricuspid valve surgery were excluded. Demographic, clinical, and laboratory findings were recorded and the relationship with progression of tricuspid insufficiency was studied. RESULTS: Valvular pathology was rheumatic in origin in 72.6% of patients. Tricuspid insufficiency was progressed in 69 (51.1%) of patients, while it did not change in 66 (48.9%) patients. Echocardiographic parameters recorded during follow-up revealed that left ventricular end-diastolic diameter decreased, while there was no significant change in right ventricular end-diastolic diameter. Tricuspid insufficiency was directly related with right ventricular end-diastolic diameter (P < .05), while no relationship was documented with left ventricular end-diastolic diameter. There was a statistically significant correlation between postoperative progression of tricuspid insufficiency and left atrial diameter; however, no relationship was documented with age, sex, valvular disease etiology, comorbidities, or preoperative atrial fibrillation. Risk factor analysis revealed that left atrial diameter was the only prognostic factor. CONCLUSION: The indications for mild and moderate tricuspid insufficiency are still not clear for patients undergoing left-sided valvular surgery. In this study, we documented that left atrial dilatation was a prognostic factor for progression of postoperative tricuspid insufficiency. In this group of patients, presence of left atrial dilatation may be an indication for tricuspid valve intervention.

Emerging biomarker for predicting acute kidney injury after cardiac surgery: cystatin C

Background/aim: Cardiopulmonary bypass (CPB)-associated acute kidney injury (AKI) is a common situation and rapid diagnosis and risk classification are important in the prevention and management of AKI. Changes in serum creatinine (SCr) levels in the current consensus criteria do not allow clinicians to diagnose CPB-associated AKI until 48 h after surgery. Materials and methods: We conducted a prospective single center study involving 57 patients who underwent cardiac surgery with CBP to compare serum beta-trace protein (BTP), neutrophil gelatinase-associated lipocalin-2 (NGAL), and cystatin C (CysC) levels with SCr for early diagnosis of CPB associated AKI. We defined AKI according to KDIGO criteria. Results: AKI was diagnosed in 24 (42.1%) patients. Mean duration of postoperative intensive care unit stay was 4.79 (± 6.12) days for the AKI+ group and 2.15 (± 0.56) days for the AKI­ group. The average length of hospital stay was 8.13 (± 5.75) days for the AKI+ group and 7.21 (± 3.68) days for the AKI­ group, which was significantly longer in the AKI+ group (P < 0.001, P = 0.011). Unlike other biomarkers, a significant increase in postoperative CysC levels was always found in patients with AKI during follow-up when compared to patients in whom AKI did not develop (P < 0.001). Conclusion: Increase in serum CysC levels showed a significant positive correlation with increase in SCr levels. We have not seen this correlation between other biomarkers and SCr. According to our study, serum CysC was a reliable biomarker that may aid in the early detection and follow-up of AKI after cardiac surgery.

Unusual presentations of Carney Complex in patient with a novel PRKAR1A mutation.

Carney Complex (CNC) is a multiple neoplasia syndrome characterized by skin tumors and pigmented lesions, myxomas, and various endocrine tumors. The aim of this case report was to describe a case of CNC with a novel PRKAR1A mutation. A man aged 46 years with a medical history of surgery for cardiac myxomas at the age of 39 was admitted to our hospital because of four newly-developed heart masses. The histologic examination confirmed cardiac myxomas. He had many presentations of CNC such as growth hormone (GH) and prolactin (PRL)-secreting mixed pituitary adenoma, benign thyroid nodule, large-cell calcifying Sertoli cell tumor (LCCST), and superficial angiomyxoma. A bilateral adrenalectomy was performed because the laboratory findings suggested primary pigmented nodular adrenocortical disease (PPNAD). The pathologic examination revealed a focal unilateral PPNAD, unilateral nonpigmented adrenocortical nodule, and bilateral adrenal medullary hyperplasia. Two years after the second cardiac operation, an interatrial septum-derived tumor was detected. An atrial myxoma was confirmed with histologic studies. Based on these findings, the patient was confirmed to have CNC. A novel insertion mutation in the type 1A regulatory subunit of the cAMP-dependent protein kinase A gene (PRKAR1A) in exon 2 was detected in our patient through genetic analysis. The presence of multiple myxomas and endocrine abnormalities should be an indication to physicians to further investigate for CNC. Herein, we described a case of CNC with a novel mutation in exon 2 of the PRKAR1A gene with typical and atypical clinical features.

Histologic alterations in tetralogy of Fallot.

BACKGROUND: This study was performed to investigate the pre-existing histologic alterations at the time of complete repair in patients with tetralogy of Fallot (TOF) and evaluate their effects on the early postoperative outcomes. METHODS: Fourteen patients, seven with acyanotic TOF (SO2 > 90, group I) and seven with cyanotic TOF (SO2 < 90, group II), undergoing complete repair, were enrolled. Right ventricular biopsies were examined for cardiomyocyte injury and fibrosis by light microscopy and mitochondrial injury by electron microscopy. The association of the severity of histologic alterations and postoperative inotrope use, intensive care unit, and in-hospital stays were evaluated. RESULTS: Compared with group I, patients in group II had a higher inotrope score (p = 0.03) and longer intensive care unit (p = 0.01) and in-hospital stays (p = 0.04). Cardiomyocyte injury and mitochondrial damage scores were higher in group II (p = 0.01 and p = 0.02, respectively). Fibrosis was detected in all specimens but was more severe in group II (p < 0.001). However, we could not demonstrate any correlation between histologic alterations and early surgical outcomes. The history of spell was significantly associated with worse early surgical outcomes (p < 0.05). CONCLUSIONS: Pre-existing cardiomyocyte injury accompanied by mitochondrial damage and fibrosis were more pronounced in cyanotic TOF patients. Early repair may prevent the development of histopathologic alterations in these patients.

Comparison of risk scoring systems in congenital heart surgery.

Hacettepe University Faculty of Medicine Department of Cardiovascular Surgery; data of 413 patients under 18 years old who had congenital heart surgery were evaluated between 01.01.2011-30.10.2012 and risk scorings were made by "RACHS-1 (Risk Adjustment in Congenital Heart Surgery)", "Aristotle Basic Complexity Score (ABS)" and "Aristotle Comprehensive Complexity Score (ACS)" systems used in evaluation of the risk of mortality and morbidity in (ACS)" systems used in evaluation of the risk of mortality and morbidity in congenital heart surgery. Data obtained were compared by Mann-Whitney U test and how effective these systems were in evaluating mortality and morbidity and their superiorities over each other were examined. Following the research, it was observed that all three systems were effective in evaluating mortality and morbidity; the most successful of them in foreseeing the event was the ACS system, ACS system was followed by RACHS-1 system and ABS system was the most incapable of these in foreseeing the event. It was seen that in detecting time related mortality, rating of these tests changed, RACHS-1 system detected the time related mortality best. It was also seen that with RACHS-1 system, a previously evaluated concept, it was possible to determine morbidity.

A giant hydatid cyst atypically located in the left ventricle.

Cardiac hydatidosis is a rare but potentially life-threatening infestation. Cardiac hydatid cysts generally occur in the left ventricle; followed by the atria, the free wall of the right ventricle, the pericardium and the interventricular septum. Herein, we report a 17-year-old girl with a giant left ventricle cyst who was previously treated by interventional methods for liver and lung hydatid cysts. The cardiac cyst was removed with cardiopulmonary bypass support.

Anomalous Origin of Left Internal Thoracic Artery from the Second Intercostal Artery / Origen Anómalo de la Arteria Torácica Interna Izquierda desde la Segunda Arteria Intercostal

Left internal thoracic artery (LITA) has increased importance and common use in coronary bypass grafting because of long-term better patency rate and improvement in both the duration and quality of survival of patients. LITA is a branch of the first part of the subclavian artery. We report a case in which the LITA originated from the second left intercostal artery. A 71-year-old man with a history of angina pectoris for 1 month was admitted to the hospital because of progressive increase in anginal symptoms. During the operation, it was noted that the LITA originated from the second intercostal artery. LITA used as a free graft. LITA was anastomosed to the left anterior descending coronary artery. He was free of symptoms 4 weeks after operation. To our knowledge, this is the first reported case of the LITA originating from the second intercostal artery.

La arteria torácica interna izquierda (ATII) ha aumentado en importancia en su uso como injerto de bypass coronario, debido a su mayor tasa de permeabilidad a largo plazo, y mejora tanto en la duración y la calidad de la supervivencia de los pacientes. La ATII es una rama de la primera parte de la arteria subclavia. Se presenta un caso, en el cual la ATII se originaba desde la segunda arteria intercostal izquierda. Un hombre de 71 años de edad, con antecedentes de angina de pecho durante 1 mes, fue ingresado en el hospital debido al aumento progresivo de los síntomas de angina. Durante la operación, se observó que la ATII se originaba a partir de la segunda arteria intercostal. La ATII fue utilizada como injerto libre, anastomosada a la arteria coronaria izquierda descendente anterior. El paciente se mostró libre de síntomas 4 semanas después de la operación. Este es el primer caso clínico reportado de la ATII originada desde la segunda arteria intercostal.

Paget-Schroetter syndrome forerunning the diagnoses of thoracic outlet syndrome and thrombophilia.

Reported here is a 22-year-old professional wrestler who was diagnosed to have Paget-Schroetter syndrome after Greco-Roman wrestling. On substantial neuromuscular examination and laboratory testing, he was found to have also thoracic outlet syndrome and heterozygous mutations for factor V Leiden and methyltetrahydrofolate reductase genes. To the best knowledge of the authors, the concomitance of these pathologies is discussed for the first time in the literature.

[Endovascular treatment of aortic lesions using the Medtronic Talent system: single center experience with mid-term follow-up]. / Aortik lezyonlarin Medtronic Talent stent-greft ile endovasküler tedavisi: Tek merkez deneyimi ve orta dönem takip sonuçlari.

OBJECTIVE: The aim of this retrospective study is to investigate the safety and efficacy of endovascular repair of aortic lesions with the Medtronic Talent stent-graft system and to present mid-term results of endovascular aortic repair performed in our center. METHODS: Between December 2002 and March 2007, 54 patients (6 women) with aortic (14 thoracic and 40 abdominal) lesions underwent treatment with Talent stent-graft. The average age of the patients was 64.8 (20-88) years. Duration of follow-up period ranged from 1 to 49 months (average 21 months). Indications for endovascular repair were degenerative aneurysm in 45, degenerative aneurysm and penetrating ulcer in 2, only penetrating ulcer in 1, traumatic thoracic isthmic transsection in 4 and vasculitic aneurysm secondary to Behcet's disease in 2 patients. RESULTS: Repair was performed with the tubular (16), aortouniiliac (1) or bifurcated (37) stent-grafts. Technical success rate was 100%. No death, major complication or need of immediate conversion to open repair was seen. Endoleak rate was 18.5% at 1 month follow-up period. Thirty-day mortality was 1.8% and morbidity (other than endoleaks) rate was 12.9%. During the follow-up period, secondary intervention was required in 12.9% of patients. Iliac limb occlusion was detected in 1 patient (1.8%). Graft migration causing type 1 endoleak and requiring open surgical treatment was seen in 1 patient (1.8%). Four patients (7.4%) are still under follow-up for type-2 endoleaks that do not require intervention. No graft infection or death due to aneurysm rupture was detected. CONCLUSION: Endovascular treatment of aortic lesions in selected patients with comorbid conditions using the Talent stent-graft exhibits a high degree of technical success with a low perioperative morbidity and mortality rate. The major disadvantage of endovascular aortic repair is necessity of life-long imaging follow-up and secondary interventions.

The role of surface ECG and transthoracic echocardiography for predicting postoperative atrial fibrillation after coronary artery bypass surgery.

OBJECTIVE: To evaluate the roles of surface electrocardiogram (ECG) and transthoracic echocardiography (ECHO) for prediction of atrial fibrillation (AF) after coronary artery bypass grafting (CABG). METHODS: This study was conducted from 2002-2004 at the Cardiovascular Department of Hacettepe University, Ankara, Turkey. Seventy consecutive patients were enrolled in this study that underwent elective CABG. A 12-lead ECG was recorded one day before cardiac surgery and was repeated during the 5 days after CABG. P-wave dispersion (PWD) was defined as the difference between maximum and minimum P-wave duration. Differences in P-wave duration were compared between the pre- and postoperative 12-lead ECG measurements. RESULTS: Postoperative AF developed in 17 (24%) cases of 70 patients. The PWD was found to be significantly higher in patients with AF preoperatively (60+/-19 versus 47+/-13, p=0.003), postoperative first day (56+/-12 versus 44+/-11, p<0.002) and fifth day (51+/-29 versus 41+/-11, p<0.001). Patients with AF were significantly older, the mean age of the AF group was (68+/-7) years and of the sinus rhythm (SR) group was (59+/-10 years) (p<0.001). The AF group had left ventricular systolic dysfunction (56+/-13% versus 56+/-8%, p=0.042, preoperatively; 49+/-8% versus 60+/-10%, p=0.001, postoperatively) and a larger left atrium (46+/-5 versus 39+/-5 mm, p<0.001, preoperatively and 44+/-7 versus 39+/-5 mm, p=0.046, postoperatively) than the SR group. CONCLUSION: This prospective study demonstrated that PWD on surface ECG and additional echocardiographic parameters are simple and reliable indexes to predict the development of AF after CABG.

Preoperative fibrinogen levels as a predictor of postoperative bleeding after open heart surgery.

BACKGROUND: Open heart surgery still involving major bleeding continues to be a major challenge after cardiac surgery and is also a significant cause of morbidity and mortality. Most hemostatic factors are intercorrelated with postoperative bleeding, and fibrinogen seems the most fundamental hemostatic risk factor for open heart surgery. METHODS: The study included 97 patients who underwent elective coronary artery surgery (78 men and 19 women; mean age, 60.9 +/- 10.3). Preoperative blood samples were obtained and preoprative quantitative determination of plasma fibrinogen levels were measured by the clotting method of Clauss using the fibrinogen kit. Patients were operated on by the same team and the same technique. The total amount of drainage blood from chest tubes was recorded after termination of operation. RESULTS: There were statistical significance between the fibrinogen levels and the drainage (r = -0.897, P < .001). Chest drainage was a mean of 972 mL (range, 240-2445 mL) in the first 48 hours after sternotomy closure. Fibrinogen level and relation to age was statistically significant (P = .015). There was no statistical significance between fibrinogen levels and gender (male gender = 400.7 +/- 123.0 versus female gender = 395.6 +/- 148.1; P = .877) and between drainage and gender (male gender = 968.2 +/- 538.5 versus female gender = 990.0 +/- 554.7; P = .876). Two patients (2%) died early after the surgery. There were no significant differences between the postoperative bleeding and cardiopulmonary bypass time (P = .648) or cross-clamp time (P = .974). CONCLUSION: The results of this study suggested that low preoperative fibrinogen level appears to be a useful diagnostic marker to assess the activity of the coagulation system, and that its preoperative level may serve as a potential risk factor for postoperative bleeding after coronary artery bypass surgery.

Predictive significance of plasma levels of interleukin-6 and high-sensitivity C-reactive protein in atrial fibrillation after coronary artery bypass surgery.

BACKGROUND: Postoperative atrial fibrillation (AF) plays a major role in the determination of hemodynamic deterioration and can be associated with cardiovascular events after coronary artery surgery. Elevated interleukin (IL)-6 and C-reactive protein (CRP) levels in patients with AF suggest a role of inflammation in the pathogenesis of AF. We conducted a study to investigate the correlation between postoperative AF and IL-6 and high-sensitivity CRP (hsCRP). MATERIALS AND METHODS: Forty-nine patients with a mean age of 60.3 +/- 10.7 years were enrolled in this study. Preoperative and postoperative first day blood samples were collected to assess the IL-6 and hsCRP levels. IL-6 levels were measured by enzyme-linked immunosorbent assay, and hsCRP was measured by rate turbidimetry method. RESULTS: Fourteen patients (28.5%) developed AF postoperatively. Patients who developed AF showed elevated serum concentrations of postoperative first day IL-6 (P < .001), preoperative hsCRP (P < .005), and postoperative first day hsCRP (P < 0.001). Preoperative hsCRP levels (P < .002) and postoperative first day IL-6 (P < .001) and hsCRP (P < 0.001) levels were associated with prolonged endotracheal intubation time. Prolonged intensive care unit stay showed significant correlations with elevated levels of preoperative hsCRP (P < 0.002) and postoperative first day IL-6 (P < 0.001) and hsCRP (P < 0.001). There was also statistical significance between the AF+ and AF- groups regarding intensive care unit stay and endotracheal intubation times (P < .001 and P < .001, respectively). Cut-off points for postoperative first day IL-6, preoperative hsCRP, and postoperative first day hsCRP were 46.4 pg/mL (sensitivity = 92.9% and specificity = 80%), 0.46 mg/L (sensitivity = 71% and specificity = 75%), and 17.9 mg/L (sensitivity = 92.9% and specificity = 78%), respectively. CONCLUSIONS: Elevated IL-6 and hsCRP levels in patients with postoperative AF suggest inflammatory components have a role of in the pathogenesis of AF.

Surgical management of infants with isolated supravalvular pulmonary stenosis: case reports.

Pulmonary stenosis (PS) can be seen from the right ventricular outflow tract to the peripheral pulmonary arteries. Most frequently, the obstruction occurs at the level of the pulmonary valve; however, it occurs less frequently at the infindibular level within the trabecular component of the right ventricle or within the pulmonary arterial pathways. Lesions at any of these levels can occur as part of more congenital cardiac malformations such as tetralogy of Fallot, complete transposition of great arteries, or atrial septal defect. Isolated supravalvular pulmonary stenosis (iSPS) is less common than other types of PS. In this study, we present our experience with 4 patients who underwent cardiopulmonary bypass operation for iSPS. In one patient, the circular stenotic area was noted on the touch point of the pulmonary valve. Right ventricular pressures ranged from 70 to 90 mmHg, and the pulmonary artery mean pressures ranged from 14 to 17 mmHg. In all patients, the left ventricular and aortic systolic, diastolic, and mean pressures were moderately increased. Pulmonary artery stenosis was treated successfully using a pericardial or Dacron patch on cardiopulmonary bypass. Various techniques such as balloon dilation have been proposed to deal with this problem, but these may often be unsuccessful because of the elasticity and recoil of the pulmonary artery constrictive ring. Even though endovascular stenting and/or balloon angioplasty have been recently proposed as an initial treatment strategy, they may be associated with some severe complications including pulmonary artery thrombosis or stent migration. Our study, even though it consists of a limited number of cases, suggests that open heart surgery using an oval-shaped patch may be a used as the other main choice for the treatment of iSPS.

Increments in circulating homocysteine before and after coronary artery bypass surgery in diabetic and non-diabetic patients.

This study aims to assess total plasma homocysteine levels in diabetic and non-diabetic patients who underwent coronary artery bypass grafting (CABG) and to determine the effect of cardiopulmonary bypass (CPB) on homocysteine levels. Twenty diabetic and twenty non-diabetic patients who had CABG were enrolled in the study. Plasma samples for the assays were obtained before and after the CPB and on sixth day following the operation. Diabetic patients seemed to have higher levels of plasma total homocysteine in pre-CPB, post-CPB, and postoperative sixth day blood samples. There was a significant difference in total homocysteine levels between pre-CPB and post-CPB both in diabetic and non-diabetic patients. Although an association between the preoperative plasma total homocysteine levels and the frequency of 1-year graft occlusion could not be demonstrated, our data claims attention to the increased homocysteine levels during CPB, which sustained until the sixth day after the surgery.

Surgical management of infants with congenital lobar emphysema and concomitant congenital heart disease.

OBJECTIVE: Congenital lobar emphysema (CLE) is an uncommon cause of infantile respiratory distress. It is diagnosed on the basis of evidence of lobar overaeration, mediastinal shift, and compression of the adjacent lobe. Concomitant congenital heart disease (CHD) and CLE is not uncommon. In the literature a 12% to 20% concomitance rate is given. The optimal treatment of respiratory symptoms associated with CLE and CHD is not clear; however, there has been a great deal of progress in the treatment of CLE and CHD. The aim of this study was to evaluate a clinical experience with and long-term follow-up of the surgical treatment of 13 patients with concomitant CLE and CHD. MATERIAL AND METHODS: We reviewed the cases of 13 patients with concomitant CLE and CHD. The medical records were evaluated with reference to age, type of CHD, pulmonary artery pressure, clinical symptoms, and results of surgical management. RESULTS: One patient died. This patient had ventricular septal defect (VSD) and left upper lobe emphysema in the postoperative period. The remaining patients undergoing follow-up were clinically well at the final evaluation. Postoperative thoracic computed tomography revealed complete spontaneous regression of emphysema 3 months after division of ductus arteriosus in 1 patient. Pulmonary hypertensive episode was seen in 3 patients after the early postoperative period. Five of the patients were discharged with bronchodilator treatment after surgery. Six patients needed positive inotropic support. Among the patients with pulmonary hypertension and those with VSD who had undergone cardiopulmonary bypass, we found a greater need for inotropic support, a higher risk of postoperative infection, and a longer intubation period. Echocardiography in the late postoperative revealed decreased pulmonary artery diameter and pressure; myocardial performance was normal. Results of blood gas analyses revealed increased oxygen saturation and decreased partial pressure of carbon dioxide. Normal exercise activity was found in all patients. DISCUSSION: The presence of CHD, especially in infants with unusual respiratory distress symptoms, should be kept in mind, and echocardiography and/or cardiac catheterization should be considered in the diagnosis. In patients with high pulmonary artery pressure, palliative or corrective surgery for CHD in addition to lobectomy can be considered. We believe that for lesions without high pulmonary artery pressure, such as small atrial septal defect and patent foramen ovale, clinical follow-up is sufficient treatment after lobectomy. If the cause of CLE is compression of large ductus arteriosus, only division of the patent ductus arteriosus may be considered before lobectomy and clinical and radiologic follow-up. The cardiac lesion should be assessed as to severity and ease of management. A corrective procedure can be carried out at lobectomy. Because of the technical ease with which the cardiac operation can be performed at the time of lobectomy, we suggest that in addition to lobectomy, operative treatment of cardiac lesions be performed.

LEOPARD syndrome and hypertrophic obstructive cardiomyopathy: a case report.

The LEOPARD syndrome is a rare, autosomal dominant multisystemic disorder characterized by lentiginosis, ocular hypertelorism, abnormal genitalia, growth retardation, sensorineural deafness, and cardiac and electrocardiographic abnormalities. Although it is not cited, hypertrophic cardiomyopathy is often associated with the disease. In this study, we present a nine-year-old boy with LEOPARD syndrome and hypertrophic obstructive cardiomyopathy.

Vascular anomalies causing tracheoesophageal compression: a 20-year experience in diagnosis and management.

BACKGROUND: Vascular rings and other congenital aortic arch anomalies may be major causes of tracheoesophageal obstruction in children. In this report, our diagnostic approach, surgical treatment, and early and late results for 30 patients are reported. METHODS: During a 20-year period (1982-2002), 30 children underwent surgery for tracheoesophageal compression caused by aortic arch anomalies. The median age at operation was 8 months (range, 36 days to 94 months), and the median patient weight was 8 kg (range, 2.4-16 kg). At 53.3% of cases, double aortic arch was by far the most common encountered cause of compression. Patients were admitted with respiratory distress, stridor, apnea, dysphagia, or recurrent respiratory tract infections. Diagnosis was established by barium esophagogram, computed tomography, magnetic resonance imaging, and angiography. The operative approaches were through a left thoracotomy or a median sternotomy. RESULTS: Operative mortality rate was 3.3%. Follow-up data from 2 months to 10 years (mean follow-up, 34 weeks) were available for all 30 patients. Twenty-six patients (86.7%) were essentially free of symptoms, 3 patients (10%) had residual respiratory problems, and 1 patient (3.3 %) had a gastroesophageal reflux problem. CONCLUSION: These results suggest that surgical correction of symptomatic vascular rings can be performed with low mortality and morbidity rates.