Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report.

BACKGROUND: Cornelia de Lange syndrome (CdLS) is a congenital multisystemic genetic disorder. The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent years. However, they still more frequently undergo cardiac surgery. There are some challenges for clinicians when faced with CdLS patients. We present the perioperative management of a child with CdLS undergoing open-heart surgery. CASE SUMMARY: Severe pulmonic and subpulmonic valvular stenosis, enlargement of the right side of the heart, mild tricuspid regurgitation, atrial septal defect, and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis, developmental delay, and malnutrition. Attempted balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve, therefore it was decided to perform an open surgical repair. Following a successful and uncomplicated intraoperative course, the patient was extubated on postoperative day 5, and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10, respectively. Moderate laryngomalacia and suboptimal vocal cord movements were diagnosed, and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day 32. The patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional problems. CONCLUSION: This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.

Effects of Sevoflurane and Propofol Anesthesia on Cerebral Oxygenation in Patients Undergoing Carotid Endarterectomy.

AIM: To compare the effects of sevoflurane and propofol anesthesia on perioperative cerebral oxygenation in patients undergoing carotid endarterectomy (CEA) under general anesthesia by using near-infrared spectroscopy (NIRS) monitoring. MATERIAL AND METHODS: Institutional approval was obtained, and the perioperative data of 33 patients undergoing CEA were retrospectively evaluated. The study groups were organized according to the anesthesia drugs used for maintenance: sevoflurane (n=17) and propofol (n=16). The regional cerebral oxygen saturation (rScO < sub > 2 < /sub > ) of the ipsilateral and contralateral hemispheres was monitored continuously using a NIRS instrument and analyzed at specific time points starting from induction to the 12th hour postoperation. The data were analyzed using the appropriate tests, and a p value of < 0.05 was considered significant. RESULTS: Compared with those of groups with non-clamped hemispheres, the rScO < sub > 2 < /sub > values of the sevoflurane and propofol groups decreased significantly during clamping (p < 0.05) and increased to above-preoperative values after declamping (p < 0.05). When the sevoflurane and propofol groups were compared, a significant decrease in rScO < sub > 2 < /sub > was noted during extubation in the sevoflurane group (p < 0.05). In the propofol group, female patients had significantly lower rScO < sub > 2 < /sub > values compared with male patients during clamping of the carotid artery (p < 0.05). None of the observed decreases was greater than 20%, which is considered an indication for shunting. CONCLUSION: Our NIRS monitoring results indicate that sevoflurane or propofol anesthesia does not generally cause significant differences in cerebral oxygenation during and after cross-clamping in patients undergoing CEA. The finding of sex-related differences in cerebral oxygenation in patients receiving propofol and decreased oxygenation during extubation in patients receiving sevoflurane warrants further studies.

Endocarditis infecciosa de la válvula tricúspide asociada con meningitis aséptica: presentación infrecuente en una niña / Tricuspid valve infective endocarditis associated with aseptic meningitis: a rare presentation in a child

La endocarditis infecciosa es infrecuente pero potencialmente mortal. Las presentaciones atípicas retrasan el diagnóstico. El compromiso neurológico es habitual en la endocarditis de la válvula mitral, aunque infrecuente en la endocarditis de la válvula tricúspide. Si bien se han informado algunos casos e el del lado derecho con síntomas neurológicos en adultos, en la bibliografía no se ha descripto en niños. Se presenta una niña de 9 años con comunicación interventricular (CIV) congénita con fiebre, cefalea y rigidez de nuca. Sus síntomas clínicos y los hallazgos en el líquido cefalorraquídeo respaldaron el diagnóstico de meningitis aséptica. El día 3 del tratamiento con ceftriaxona, se resolvieron los síntomas; tras nueve días, reingresó con fiebre y rigidez de nuca. Un ecocardiograma mostró endocarditis de la válvula tricúspide. Recibió tratamiento antibiótico durante 6 semanas. Se realizó una cirugía cardíaca para la CIV y la insuficiencia de la válvula tricúspide.

Infective endocarditis (IE) is a rare but a potentially life-threatening infectious disease. Atypical presentations cause delays in the diagnosis. Neurological involvement such as meningitis or meningismus, are especially common in mitral valve endocarditis, but unusual in tricuspid valve endocarditis. Although few cases of right-sided IE have been reported with neurological symptoms in adults, children have not been described in literature. A nine-year-old girl with congenital ventricular septal defect (VSD) was admitted with fever, headache and neck stiffness. Her clinical symptoms and cerebrospinal fluid findings supported the aseptic meningitis. On ceftriaxone therapy day 3, her complaints were resolved; nine days later she was admitted with fever and neck stiffness again. Further investigation for fever source with echocardiogram revealed a tricuspid valve endocarditis. Antibiotic therapy was completed after 6 weeks. Cardiac surgery was performed for VSD and tricuspid valvular insufficiency.

Tricuspid valve infective endocarditis associated with aseptic meningitis: a rare presentation in a child. / Endocarditis infecciosa de la válvula tricúspide asociada con meningitis aséptica: presentación infrecuente en una niña.

Infective endocarditis (IE) is a rare but a potentially life-threatening infectious disease. Atypical presentations cause delays in the diagnosis. Neurological involvement such as meningitis or meningismus, are especially common in mitral valve endocarditis, but unusual in tricuspid valve endocarditis. Although few cases of right-sided IE have been reported with neurological symptoms in adults, children have not been described in literature. A nine-year-old girl with congenital ventricular septal defect (VSD) was admitted with fever, headache and neck stiffness. Her clinical symptoms and cerebrospinal fluid findings supported the aseptic meningitis. On ceftriaxone therapy day 3, her complaints were resolved; nine days later she was admitted with fever and neck stiffness again. Further investigation for fever source with echocardiogram revealed a tricuspid valve endocarditis. Antibiotic therapy was completed after 6 weeks. Cardiac surgery was performed for VSD and tricuspid valvular insufficiency.

La endocarditis infecciosa es infrecuente pero potencialmente mortal. Las presentaciones atípicas retrasan el diagnóstico. El compromiso neurológico es habitual en la endocarditis de la válvula mitral, aunque infrecuente en la endocarditis de la válvula tricúspide. Si bien se han informado algunos casos e el del lado derecho con síntomas neurológicos en adultos, en la bibliografía no se ha descripto en niños. Se presenta una niña de 9 años con comunicación interventricular (CIV) congénita con fiebre, cefalea y rigidez de nuca. Sus síntomas clínicos y los hallazgos en el líquido cefalorraquídeo respaldaron el diagnóstico de meningitis aséptica. El día 3 del tratamiento con ceftriaxona, se resolvieron los síntomas; tras nueve días, reingresó con fiebre y rigidez de nuca. Un ecocardiograma mostró endocarditis de la válvula tricúspide. Recibió tratamiento antibiótico durante 6 semanas. Se realizó una cirugía cardíaca para la CIV y la insuficiencia de la válvula tricúspide.

A rare giant pericardial cyst mimicking a paracardiac mass.

Pericardial cysts are rare benign lesions of the thoracic cavity and are mostly congenital anomalies. They are induced by an incomplete coalescence of foetal lacunae during the development of the pericardium. Pericardial cysts are usually unilocular, well marginated spherical or teardrop shaped and may be attached to the pericardium directly or by a pedicle. Of all pericardial cysts, 70 to 75% are located at the right cardiophrenic angle. We report a case that was incidentally diagnosed with only chest magnetic resonance imaging because of a paracardiac mass. In order to prevent complications, the giant pericardial cyst was excised outside of the pericardium with median sternotomy.

Renal effects of coronary artery bypass graft surgery in diabetic and non-diabetic patients: a study with urinary neutrophil gelatinase-associated lipocalin and serum cystatin C.

BACKGROUND/AIMS: Cardiac surgery and diabetes are major causes of acute kidney injury (AKI). We aimed to investigate the value of urinary neutrophil gelatinase-associated lipocalin (NGAL) and serum cystatin C, as early biomarkers for prediction of AKI in diabetic and non-diabetic adult patients undergoing cardiac surgery. METHODS: 15 non-diabetic and 15 diabetic adult patients undergoing cardiac surgery were enrolled. Peri-operative clinical and laboratory variables were recorded. Urinary NGAL, serum cystatin C, serum creatinine (Cre) and blood urea nitrogen (BUN) were evaluated. RESULTS: AKI was detected in 4 patients in non-diabetic group and 12 patients in diabetic group. Urinary NGAL levels of diabetic and non-diabetic patients and serum cystatin C levels of diabetic patients exceed upper normal limits or cutoff values much earlier than BUN and Cre. cystatin C levels of non-diabetic patients remain unchanged. Cystatin C and NGAL levels of patients meeting AKI criteria and NGAL levels of patients not meeting AKI criteria exceeded upper normal limits or cut off values much earlier in than BUN and Cre. CONCLUSIONS: Measurement of cystatin C level in both diabetic and non-diabetic patients may reveal AKI earlier than NGAL and BUN. In diabetic patients, measurement of urinary NGAL and serum cystatin C levels may indicate AKI signs earlier than BUN and Cre.

Anesthetic management of a newborn with trisomy 18 undergoing closure of patent ductus arteriosus and pulmonary artery banding.

BACKGROUND: Peri-operative management of infants with trisomy 18 syndrome is challenging due to various congenital cardiac and facial anomalies. CASE REPORT: We report the anaesthetic management of a 13-day-old neonate with 1 540 g body weight, undergoing closure of patent ductus arteriosus and pulmonary artery banding. Anaesthesia was induced with sevoflurane, fentanyl and rocuronium. Despite dysmorphic facial features, ventilation and endotracheal intubation were achieved uneventfully. Anaesthesia was maintained with sevoflurane and fentanyl and was uneventful. The patient was transferred to the neonatal ICU intubated and with ventilatory support. The baby was extubated on the second day postoperatively. CONCLUSION: Our knowledge of the proper anaesthetic technique for children undergoing palliative or corrective surgery is limited. Further case reports will increase our experience in peri-operative management of children with trisomy 18.

Endovenous laser ablation under general anesthesia for day surgery: feasibility and outcomes of the 300 patients.

PURPOSE: Endovenous laser ablation (EVLA) for superficial venous insufficiency is traditionally performed under tumescent local anesthesia as day case surgery. The aim of this study is to evaluate the feasibility of general anesthesia in addition to tumescent anesthesia in patients undergoing EVLA. METHODS: The anesthesia and clinical registration records of 341 extremities of 300 adult patients were reviewed and analyzed retrospectively. Demographic and clinical data, preoperative anesthetic evaluation data (ASA physical status, preoperative airway assessment, Mallampati score), type of supraglottic device, duration of anesthesia and surgery, any surgical and/or anesthetic complication, timing of mobilization and discharge, and postoperative course were evaluated. RESULTS: Mean duration of operation and anesthesia was 28 (12-55) and 40 (20-65) minutes, respectively. Mobilization and discharge timing was 25 (11-45) and 139 (110-200) minutes, respectively. All patients were discharged the same day of surgery. CONCLUSION: The combination technique of administering general anesthesia with supraglottic device and tumescent anesthesia is a safe and effective method to reduce the patients' pain and discomfort during the EVLT procedure within the scope of day case surgery.

Cardiac angiosarcoma: A case with bilateral pleural and pericardial effusion.

Cardiac sarcomas are rare malignant tumors. Angiosarcoma is the most common cardiac sarcoma and is present in up to 33% of cases. Angiosarcomas have a poor prognosis, with a short survival expectancy. We report a case of a right atrial angiosarcoma treated by partial tumor resection followed by chemotherapy.

Extremely low birth weight infants with patent ductus arteriosus: searching for the least invasiveness.

Patent ductus arteriosus (PDA) is an important problem in premature infants. Extremely low birth weight infants (ELBWI) are so fragile with respect to surgical stress that minimally invasive procedures are required. We report 26 ELBWI cases with PDA who underwent surgical closure. All had failed indomethacin treatment, or it had been contraindicated. The mean gestational age at birth was 27 weeks (range, 24-38 weeks), and the mean birth weight was 960.96 g (range, 710-1440 g). The mean age at operation was 18.06 days (range, 7-34 days), and the mean body weight at operation was 989.42 g (range, 680-1460 g). There was no surgery-related mortality or morbidity. Our surgical procedures consisted of posterior muscle-sparing thoracotomy, clipping the PDA and no ligation, and closing the thorax without a tube thoracostomy. Muscle-sparing thoracotomy reduces the likelihood of long-term physical impairment and deformity, the clipping technique minimizes the dissection of surrounding PDA tissue, and the thorax is closed without a tube. Nursing care is simplified, costs are reduced, and the number of chest x-rays needed postoperatively is reduced. We believe that surgical closure of PDA without chest tube drainage can be accomplished safely in premature infants.

Spontaneous coronary artery dissections: three case reports and review of literature.

Primary spontaneous coronary artery dissection is one of the rare causes of acute myocardial infarction and is mostly fatal. Previous studies report that it is mostly seen in middle-aged women in the last trimester of pregnancy and early postpartum period. Clinical presentation of the disease is variable in pattern, and its severity is related to extent and development rate of dissection. Herein we present three cases of primary spontaneous left main coronary artery dissection. Two of the patients are men and the third patient is a non-pregnant 69-year-old woman. The cases were presented and discussed with review of the pertinent literature.

Surgical treatment of right ventricular hydatid cyst.

Hydatid cyst is a serious endemic parasitic disease found in cattle-raising areas of the world. Cardiac hydatid cysts are rare and appear in 0.5% to 2% of hydatid cyst cases. A 24-year-old male patient was admitted to the hospital because of chest pain. A cystic mass (4 4 3 cm) was demonstrated with transthoracic echocardiography, computed tomography, and magnetic resonance imaging. A hydatid cyst was located in the right ventricular wall near the inferior branch of the acute marginal branch of the right coronary artery and was located such that it pushed the tricuspid valve inward. The cystic materials were removed with the patient on cardiopulmonary bypass. The surgery for cardiac hydatid disease is safe, and the results are satisfactory.

Plasma leptin levels increase to a greater extent following on-pump coronary artery surgery in type 2 diabetic patients than in nondiabetic patients.

AIMS: We aimed to evaluate whether leptin and ghrelin responses to cardiopulmonary bypass (CPB) are dependent on type 2 diabetes and whether these responses are associated with interleukin-6 (IL-6), high-sensitivity C-reactive protein (hsCRP), cortisol and insulin. METHODS: We examined stress-response patterns in plasma leptin, ghrelin, hsCRP, IL-6, cortisol and insulin levels before and up to 5 days after cardiopulmonary bypass in 20 patients with type 2 diabetes and 20 patients without diabetes. RESULTS: Plasma leptin levels increased significantly in both groups (p<0.05) and rose significantly higher in diabetics when compared with nondiabetic patients (p=0.004). Plasma ghrelin levels increased significantly only in diabetics (p=0.033). Patients with and without diabetes showed significantly elevated serum concentrations of IL-6, hsCRP, cortisol and insulin (p<0.005 for IL-6, hsCRP; p<0.05 for cortisol, insulin) but the difference between the two groups was nonsignificant. Leptin was independently predicted by hsCRP (p<0.05, F=2.9), gender (women p<0.001, F=4.7), body mass index (BMI p<0.0001, F=6.1) whereas ghrelin levels were not associated with any variables in the total patient population. (critical F=2.26, p≤0.05). CONCLUSIONS: Acute phase response in diabetics differs by higher leptin levels independent of BMI, gender and IL-6, hsCRP, insulin and cortisol levels.

Peroperative effects of fresh frozen plasma and antithrombin III on heparin sensitivity and coagulation during nitroglycerine infusion in coronary artery bypass surgery.

Nitroglycerin (NTG) reduces the anticoagulant effects of heparin and may lead to heparin resistance. Fresh frozen plasma (FFP) and antithrombin III (ATIII) may be used for the treatment of heparin resistance. We aimed to compare the effects of FFP and ATIII on heparin requirement, coagulation parameters, and bleeding in patients undergoing coronary artery bypass graft surgery (CABGS) with moderate dose of intraoperative NTG infusion. Forty-eight patients undergoing CABGS with NTG infusion were randomly allocated to three groups. Group C served as control, whereas the patients in group P received FFP and those in group A received ATIII after anesthesia induction. ATIII activity and coagulation parameters were measured at five different times intraoperatively. Total heparin requirement, heparin consumption, and heparin sensitivity were calculated. ATIII activity and ACT were significantly higher and activated partial thromboplastin time and fibrinogen level were significantly lower during cardiopulmonary bypass in group A than in groups P and C. Heparin sensitivity was significantly higher and total heparin requirement and consumption were significantly lower in ATIII group than in other groups. ATIII administration increases heparin sensitivity and decreases heparin requirements compared with FFP in patients undergoing CABGS with peroperative NTG infusion. ATIII may be preferred to FFP in patients with heparin resistance due to NTG infusion undergoing CABGS.

Heat shock protein70: a new marker for subsequent atrial fibrillation development?

Atrial fibrillation (AF) is a common arrhythmia, after cardiac surgery, and it is associated with a twofold increase in cardiovascular mortality and morbidity. Reperfusion injury and inflammation associated with cardiac surgery are thought to be involved in its pathogenesis. Heat shock proteins (HSPs) are a family of chaperone proteins which assist in preservation of cellular integrity by maintaining proteins in their correctly folded state. The aim of this study was to investigate pre-postoperative heat shock protein70 (HSP70) and high-sensitivity C-reactive protein in serum from patients in preoperative sinus rhythm. We prospectively screened 45 consecutive patients admitted to the hospital for elective coronary artery bypass surgery (CABG). Electrocardiogram characteristics and cardiovascular risk profile were documented. Pre- and postoperative blood samples were collected. HSP70 value was 8.9 +/- 4.8 ng/mL in Group A (study group) preoperatively and decreased to 7.7 +/- 7.0 ng/mL postoperatively. In contrast, preoperative value of HSP70 was 4.2 +/- 2.2 ng/mL and decreased to 2.7 +/- 2.6 ng/mL postoperatively in Group B (control group). Statistical analysis showed significant difference regarding preoperative HSP70 levels in Group A compared to Group B. To our knowledge, with this study, the association of pre- and postoperative circulating HSP70 with postoperative AF was demonstrated for the first time.

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.

Isolated axillary artery injury due to blunt trauma.

The intimal damage of the axillary artery due to an acute, single blunt trauma is very rare without concomitant bone, brachial plexus, venous and soft tissue injuries. Early diagnosis and appropriate management of the arterial injury is essential to avoid permanent disability. The clinical signs are usually occult and do not become manifest until a long ischemic interval following injury, owing to the extensive collateral network. A twenty-year-old male patient had injured his left arm in a hyperabduction and hyperextension position while he was carrying a refrigerator with his arm. An increase in the intensity of pain and numbness reappeared in his left arm 1.5 months after the trauma. Digital subtraction angiography of the axillary artery performed after his hospitalization showed an occlusion of the axillary artery and no reconstitution of distal part of the occlusion via collateral vessels. During the operation, the axillary and brachial arteries were bypassed with a saphenous graft. As shown in this case report, in the early period after blunt trauma of the upper limb, progressive signs of vascular compromise may disappear because of collateral circulation even if the distal pulses are absent. Then an angiography of the upper limb becomes essential for correct diagnosis and treatment. This is our second experience. On the basis of our first experience that was reported, in such a chronic case, oral anticoagulation must be carried out at least six months whenever a graft thrombosis after revascularization is encountered.