Anesthetic considerations in a parturient with Oral-Facial-Digital syndrome and repaired tetralogy of Fallot with left ventricular dysfunction

Oral-Facial-Digital syndrome or Mohr syndrome is a rare congenital disorder characterized by malformations of face, oral cavity, laryngeal structures, trachea, and digits, muscular-skeletal abnormalities, and congenital cardiac defects. In this case report, we describe the anesthetic management of a parturient with Oral-Facial-Digital syndrome type II and repaired tetralogy of Fallot with left ventricular dysfunction.     

Anesthesia for cesarean section in a patient with Holt-Oram syndrome

Holt-Oram syndrome is a rare genetic disorder affecting the heart and upper limbs (atriodigital dysplasia). The manifestations of the limb defects may vary in severity from subtle carpal bone defects and triphalangeal thumb to digit aplasia and upper extremity phocomelia. Cardiac abnormalities include atrial and/or ventricular septal defects, anomalies in pulmonary venous return and various dysrhythmias. We present the anesthetic management of a parturient with this syndrome who underwent elective cesarean section and tubal ligation, conducted under combined spinal-epidural anesthesia with a low dose of intrathecal bupivacaine. Our goal was to avoid an excessively high sympathetic block or excessive sympathetic stimulation accompanied by potential deleterious effects on cardiac rhythm. Cardiac monitoring was continued in the postoperative period for 6 h because of the possibility of dysrhythmia.     

Anesthetic considerations in a parturient with Freeman–Sheldon syndrome

Freeman–Sheldon syndrome is a rare genetic disorder characterized by malformations of the face, oral cavity and musculoskeletal system. This case report describes the anesthetic management of a parturient with Freeman–Sheldon syndrome, kyphoscoliosis and a cardiac pacemaker for a cesarean delivery and tubal ligation. With a predicted difficult airway, our team decided to provide a combined spinal–epidural anesthetic. Problems encountered included difficult intravenous access, failure to identify the subarachnoid space and patient discomfort during surgery.     

Anesthetic management of an obstetric patient with MELAS syndrome: case report and literature review

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS syndrome) is a mitochondrial disorder associated with neurologic, cardiac, neuromuscular, hepatic, metabolic and gastrointestinal dysfunction and potential anesthetic and obstetric complications. The case of a parturient with MELAS syndrome requiring labor analgesia is presented. A Medline literature search limited to the English language was undertaken to review cases of MELAS syndrome. Based on our experience and literature review, parturients with MELAS syndrome appear to benefit from neuraxial analgesia and anesthesia, which blunt excessive oxygen consumption and acidosis.     

Caesarean section for twin pregnancy in a parturient with Brugada syndrome

Brugada syndrome is a common cause of sudden cardiac death. We report the anaesthetic management of a woman with Brugada syndrome for elective caesarean section of twins. There are few reports of this disease in pregnancy and we believe this is the first of elective caesarean section in a parturient with the syndrome. The characteristic electrocardiographic changes of the syndrome are linked to sodium channel blockers including local anaesthetics such as bupivacaine. The use of bupivacaine for intrathecal central neuraxial blockade as well as other drugs commonly used in obstetric anaesthesia is discussed.     

Spinal anesthesia for Cesarean section in a parturient with long QT syndrome

PURPOSE: To report the first use of spinal anesthesia for Cesarean section (CS) in a parturient with a long QT syndrome (LQTS) and an automatic implantable cardiac defibrillator (AICD). Although both general and epidural anesthesia have been described for CS in patients with LQTS, there are no previous case reports on the use of spinal anesthesia. The clinical features, diagnosis, treatment and anesthetic management of LQTS are discussed. CLINICAL FEATURES: A 31-yr-old woman, gravida 2 para 1 known to have LQTS and an AICD, presented in labour at 35 weeks gestation, three weeks before her scheduled CS. Her previous delivery by CS under spinal anesthesia at our institution was uneventful. On this occasion, we elected to administer spinal anesthesia because she was asymptomatic (no arrhythmia or cardiac arrest) for the last few years, was hemodynamically stable, and had received uneventful spinal anesthesia before. CONCLUSION: Spinal anesthesia was used safely for CS in this parturient with LQTS.     

General anaesthesia for caesarean section in a parturient with Noonan's syndrome

We describe the anaesthetic management of a parturient with Noonan's syndrome. Her problems included severe cardiac disease, facial abnormalities and extreme phobia to needles. After intrauterine death at 30 weeks gestation, induction of labour was attempted and extradural analgesia initiated using low-dose bupivacaine. She failed to progress and underwent Caesarean section under general anaesthesia using awake oral fibreoptic intubation.      

Postural orthostatic tachycardia syndrome: anesthetic implications in the obstetric patient

We report the anesthetic management of a parturient with postural orthostatic tachycardia syndrome. This syndrome is associated with hemodynamic instability, which can be worsened by the physiology of labor and delivery. We discuss anesthetic concerns with this disease in the parturient and suggest approaches for management of this disease.     

直立性心动过速综合征：产科麻醉处理的思考

我们报道一例患有直立性心动过速综合征产妇的麻醉处理，这种综合征表现为血流动力学不稳定，并可能随产程及分娩生理的变化而恶化。我们讨论对患有此类疾病产妇的麻醉相关问题，并提出麻醉处理的方法。     

Minimally- and non-invasive assessment of maternal cardiac output: go with the flow!

The measurement of cardiac output may be crucial in the management of the parturient with haemodynamic instability due to critical illness or cardiac disease. Invasive haemodynamic monitoring may not be desirable due to the potential risk of complications and issues with patient compliance. Minimally- and non-invasive techniques of cardiac output measurement include those based on ultrasonic technology and pulse contour waveform analysis. This review article provides a synopsis of the literature examining currently available minimally- and non-invasive techniques for maternal cardiac output monitoring and looks at their advantages and disadvantages with respect to the parturient.     

Syndrome of congenital ventricular diverticulum and midline thoraco-abdominal defects.

The long-term follow-up of a 30-year-old patient who had a correction of the syndrome of left ventricular diverticulum and thoraco-abdominal defects is presented. The main features of the syndrome include a diverticulum of the left ventricle, a ventricular septal defect, and sometimes other cardiac anomalies. The thoraco-abdominal defects consist of foreshortened sternum, pericardial and diaphragmatic defects, and umbilical hernia. The association of these anomalies is thought to be due to a developmental failure of the primitive paramidline mesoderm. The diagnosis can be made clinically by the presence of a pulsatile, epigastric mass associated with signs of cardiac septal defects and dextrocardia. The prognosis of patients with this syndrome depends mainly on the associated abnormalities although rupture of the diverticulum can be a fatal complication. The treatment of choice is resection of the diverticulum combined with repair of associated anomalies which can give good early and late results.     

General anesthesia using remifentanil for Cesarean delivery in a parturient with Marfan’s syndrome

PURPOSE: Due to cardiovascular and skeletal abnormalities, anesthetic management of parturients with Marfan's syndrome can be particularly challenging. Parturients with aortic root dilatation are at risk for aortic dissection. We describe the anesthetic management of a parturient with Marfan's syndrome and aortic root dilatation, who required general anesthesia for Cesarean delivery. CLINICAL FEATURES: At 26 weeks gestation, a nulliparous woman with Marfan's syndrome presented to the Anesthesia Clinic. Her history revealed asymptomatic aortic root dilatation of 41 mm, and partial correction of scoliosis with Harrington rods. Her cardiologist advised metoprolol, serial echocardiograms, and Cesarean delivery to decrease the risk of aortic dissection. At a multidisciplinary conference, a decision was made to proceed with Cesarean delivery, at term, at the cardiac surgery centre. After placement of arterial and central lines, general anesthesia was induced with remifentanil, propofol, and succinylcholine. Anesthesia was maintained with N(2)O, sevoflurane, and remifentanil (0.02-0.08 microg x kg(-1) x min(-1)). Transesophageal echocardiography examination confirmed stable aortic root dilatation. The patient remained hemodynamically stable. The baby's Apgars were 4 and 8, at one and five minutes, respectively. At the end of the procedure, the patient's trachea was extubated when she was awake. Initial postoperative care was in the intensive care unit. Both mother and baby recovered uneventfully. CONCLUSIONS: Peripartum hemodynamic changes can be life-threatening to the parturient with Marfan's syndrome and aortic dilatation. Anesthetic goals for delivery included preparation for possible aortic dissection, and avoidance of increased aortic root shear stress, through careful hemodynamic monitoring, and general anesthesia using remifentanil.     

Worsening of neurologic symptoms after epidural anesthesia for labor in a Guillain-Barré patient

We report the case of a pregnant woman presenting with Guillain-Barré syndrome in which her neurologic status worsened immediately after delivery under epidural analgesia. We believe that the anesthetic technique may have played a role in the disease progression. Because of this potential association, the risks of worsening neurologic status after regional anesthesia and alternative analgesia options should be discussed with the parturient. IMPLICATIONS: Because of the potential interaction between regional anesthesia and Guillain-Barré syndrome in pregnant women, the risks of worsening neurologic status and alternative analgesia options should be discussed with the parturient.     

Management of acquired cardiac disease in the obstetric patient

Physiologic changes incurred by pregnancy can cause severe decompensation in the parturient with underlying cardiac disease. The result is increased morbidity and mortality for both mother and child. Appropriate anesthetic management can significantly impact these outcomes. This review systematically presents the pathophysiology, peripartum risk, and anesthetic management in the puerperium of specific acquired cardiac abnormalities including: valvular disease, pulmonary hypertension, cardiomyopathy, cardiac transplantation, ischemia, arrhythmias, and cardiac arrest.     

Thrombocytopenia,low molecular weight heparin,and obstetric anesthesia

The parturient with coagulation defects, whether related to thrombocytopenia or to anticoagulation therapy, presents a unique challenge to the anesthesiologist. The risk of spinal or epidural hematoma in these patients has not been quantified fully but is a factor that one must consider on a case-by-case basis in determining whether neuraxial anesthesia is appropriate for the parturient. Following the guidelines set forth in this article should help reduce the risk of spinal or epidural hematoma without sacrificing the quality of care provided to patients.     

Pregnancy and the univentricular heart: case report and literature review

The univentricular heart is a rare form of congenital cardiac disease. We report the successful management of a parturient with a single ventricle, transposition of the great arteries, pulmonary hypertension and thrombosis of the superior vena cava. The univentricular heart is discussed in detail and the outcome in other mothers with similar cardiac anomalies is reviewed.     

Diversity of congenital cardiac defects and skeletal deformities associated with the Holt–Oram syndrome

INTRODUCTION

The Holt–Oram syndrome is a rare congenital disorder involving the skeletal and cardiovascular systems. It is characterized by upper limb deformities and cardiac malformations, atrial septal defects in particular.

PRESENTATION OF CASE

Four consecutive patients 1–15 years old with the Holt–Oram syndrome presented over a 10 year span for surgical treatment of their cardiac maladies. The spectrum of the heart defects and skeletal deformities encountered in these patients are described and discussed.

DISCUSSION

The Holt–Oram syndrome is an autosomal dominant condition; however absence of the morphological features of the trait in close family members is not rare. Although patients are known to predominately present with atrial septal defects, other cardiovascular anomalies, including rhythm abnormalities, are not uncommon. Skeletal disorders vary as well.

CONCLUSION

Cardiovascular disorders, skeletal malformations and familial expression of the Holt–Oram syndrome, vary widely.     

Epidural labour analgesia in a parturient with Noonan syndrome: a case report

PURPOSE: Noonan syndrome is a relatively uncommon genetic disorder with implications for anesthesia due to multiple organ system involvement. Pregnancy presents additional concerns and there are only four reported cases of anesthesia for Cesarean delivery in parturients with Noonan syndrome. We describe the first reported management of labour analgesia in a parturient with Noonan syndrome culminating in vaginal delivery. CLINICAL FEATURES: A 21-yr-old parturient with Noonan syndrome received patient-controlled epidural analgesia for labour at 39 weeks gestation. Meticulous attention to the anesthetic technique resulted in good analgesia, and a successful outcome for mother and child. The different approaches to labour analgesia in parturients, with particular attention to combined spinal epidural vs epidural analgesia in this setting are discussed. CONCLUSION: Parturients with Noonan syndrome can present with an array of anomalies that may present difficulties to the anesthesiologist including a difficult airway, cardiopulmonary abnormalities, exaggerated lumbar lordosis and short stature. Careful preoperative consultation and determination of the degree of associated anomalies will help to prepare the anesthesiologist for potential problems.     

Optimal timing of pediatric surgery to prevent deleterious effects on associated cardiac defects

Although cardiac defects are thought to have deleterious effects on the outcome of general pediatric surgery due to low cardiac output syndrome and hypoxemia, both pediatric surgery and cardiac surgery can be performed at the optimal timing with good results. However, some conditions requiring pediatric surgery may have deleterious effects on the outcome of cardiac surgery. Airway obstructive diseases sometimes require concomitant repair of the associated cardiac defects. In particular, tracheal stenosis may be repaired in cooperation with not only general pediatric and cardiac surgeons but also with pediatric thoracic surgeons who work in other healthcare institutions. Low birth-weight infants with symptomatic patent ductal artery are at risk of poor outcome. For patients with right isomerism, midgut malrotation or sliding hernia should be diagnosed early and repaired at the optimal timing to prevent urgent surgical intervention. Pediatric surgery should be performed at the optimal timing to prevent any deleterious effects on cardiac defects. Moreover, respiratory infection and neurologic disease should be treated to reduce late deaths.     

Cornelia de Lange syndrome

Cornelia de Lange syndrome (CdLS) is a rarely seen multisystem developmental disorder syndrome characterized by facial dysmorphia (arched eyebrows, synophrys, depressed nasal bridge, long philtrum, down-turned angles of the mouth), upper-extremity malformations, hirsutism, cardiac defects, growth and cognitive retardation, and gastrointestinal abnormalities. We present here a case of newborn male who presented with the complaints of feed regurgitation, choking and cyanosis. There was a distinct facial dysmorphism with arched and bushy eyebrows, long philtrum, thin upper lip, depressed nasal bridge and hirsutism. The patient was diagnosed as having Cornelia de Lange syndrome on the recognition of distinctive facial features in addition to the pre- and postnatal growth retardation, feeding problems and physical malformations including limb defects.