|Year : 2007 | Volume
| Issue : 3 | Page : 240-243
Anaesthetic management of emergency pacemaker implantation in a case of neonatal lupus erythematosus with complete congenital heart block & severe respiratory distress
Usha Kiran1, Arindam Dutta2, Alok Mehra3, Sambhu N Das4, Khalid Zuber3
1 MD, Professor & Head, Department of Cardiac Anesthesiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110029, India
2 MD* Classified Specialist, Indian Naval Hospital Ship Kalyani, Visakhapatnam - 530005, India
3 MD, Senior Resident, Department of Cardiac Anesthesiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110029, India
4 MD, Consultant, Department of Cardiac Anesthesiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110029, India
|Date of Acceptance||11-Mar-2007|
|Date of Web Publication||20-Mar-2010|
Professor & Head, Department of Cardiac Anaesthesiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110029
Source of Support: None, Conflict of Interest: None
An 8-week old 3-kilogram male baby was brought to this tertiary care hospital with respiratory distress, marked tracheal tug, poor feeding and a heart rate of 46/minute. The child had been referred from a peripheral hospital as a case of neonatal lupus with complete congenital heart block. The mother was seropositive for systemic lupus erythematosus with a history of two abortions. Evaluation on admission revealed a heart rate between 40-60/ minute, respiratory rate 40-50/ minute, inspiratory stridor, bilateral crepitations, chest retraction and a marked tracheal tug that improved with prone positioning. Electrocardiography and echocardiography confirmed complete congenital heart block with cardiomegaly and mild left ventricular dysfunction. Keeping in view the impending congestive heart failure, possible early cardiomyopathy and the bad obstetric history urgent pacemaker implantation was planned to allow early recovery of the child. The anaesthetic risk was high due to the heart block, ventricular dysfunction, laryngomalacia, severe tracheal tug and anticipated difficult weaning from controlled ventilation. General anaesthesia was administered with endotracheal tube and controlled ventilation using ketamine, rocuronium and sufentanil. For patient safety invasive monitoring was provided and external pacing was kept standby. Epicardial pacemaker leads were implanted onto the left ventricular wall through a left anterior 6th intercostal space thoracotomy. The child was electively ventilated for two post operative days. The tracheal tug and secretions gradually subsided over 2 weeks with oxygen, antibiotics, steroids, bronchodilators and physiotherapy. At the time of discharge from hospital 2 weeks after the implant the child was feeding well, tracheal tug was minimal and the lungs were clear.
Keywords: Neonatal lupus erythematosus; Complete congenital heart block.
|How to cite this article:|
Kiran U, Dutta A, Mehra A, Das SN, Zuber K. Anaesthetic management of emergency pacemaker implantation in a case of neonatal lupus erythematosus with complete congenital heart block & severe respiratory distress. Indian J Anaesth 2007;51:240-3
|How to cite this URL:|
Kiran U, Dutta A, Mehra A, Das SN, Zuber K. Anaesthetic management of emergency pacemaker implantation in a case of neonatal lupus erythematosus with complete congenital heart block & severe respiratory distress. Indian J Anaesth [serial online] 2007 [cited 2019 Sep 15];51:240-3. Available from: http://www.ijaweb.org/text.asp?2007/51/3/240/61152
| Introduction|| |
Neonatal Lupus Erythematosus (NLE) results from maternal transfer of IgG auto-antibodies, between 12th and 16th week of gestation. The principal serological markers, anti-SS-A/Ro and anti-SS-B/La maternal antinuclear antibodies, are transferred across the placenta and can be detected in the affected child for the first few months of life. Manifestations include congenital heart block (CHB), cutaneous lesions, thrombocytopenia, neutropenia, pulmonary or neurological disease and rarely hepatitis. The near-universal association with serological markers suggests that CHB is a passively acquired autoimmune disease, in which maternal autoantibodies cross the placenta and injure the previously normal fetal heart. NLE with CHB and the other clinical manifestations are grouped under the heading of neonatal lupus syndromes. This term - derived from resemblance of the neonatal rash to the sub acute cutaneous rash of systemic lupus erythematosus (SLE) in adults- is a misnomer, as the newborn does not have a systemic autoimmune disease and the mother may be completely asymptomatic. The noncardiac manifestations of NLE are generally transient, resolving at about 6 months of life coincident with the disappearance of maternal autoantibodies from the neonatal circulation. To date, however, complete(3rd degree) heart block is essentially irreversible .
We report a rare case of NLE with complete CHB, severe respiratory distress and marked tracheal tug treated with urgent pacemaker implantation and ventilatory support. The aim of this paper is to present this unusual case and highlight the challenging anaesthetic and perioperative management strategies.
| Case report|| |
An 8-week old male 3 kg baby presented with severe respiratory distress, tracheal tug, productive cough and poor feeding since birth. The child was born at term to a 27 year old mother with strongly positive prenatal antinuclear antibody (ANA), anti SS-A/Ro, anti SS-B/La antibody titres; weakly positive anti dsDNA autoantibody test and history of two previous abortions at 10 weeks gestation. Prenatal ultrasonography had shown fetal bradycardia and 2D echo/ Doppler studies showed a large ostium secondum ASD, gross RV hypertrophy with good biventricular function. A provisional prenatal diagnosis of a storage disorder (inborn error of metabolism associated with cardiomyopathy) with cardiac conduction abnormality was made. At birth the baby was 2.5 kg weight and had severe respiratory distress, tracheal tug (which improved with change of posture), cyanosis and a heart rate of 60/ minute. ECG showed complete CHB. 2D echocardiography/ Doppler studies, consistent with the prenatal findings, also showed significant RV systolic dysfunction, congestive hepatomegaly and IVC dilatation. He was immediately admitted to a neonatal intensive care unit at a primary care hospital and treated with oxygen (hood), inotrope, steroid, carnitine (125 mg BD for 7 days), antibiotics and IV fluids. He was diagnosed as a case of NLE with CHB, hypertrophic cardiomyopathy and advised immediate referral to a higher center. However the parents, unmindful of the consequences, brought the child to our tertiary care center only at 8-weeks age with a deteriorating respiratory distress.
On examination the child's heart rate was 46/min, respiration 48/min with retraction, marked tracheal tug; there were extensive, bilateral crepitations; Hb was 13.6 g%; ECG showed CHB with heart-rate of 48/min [Figure 1](A); chest-x-ray showed mild cardiomegaly [Figure 2](A) and 2D echocardiography showed CHB, no structural heart disease with mild LV dysfunction.
The child was taken up for urgent pacemaker implantation as a high risk case due to the complete heart block, possible hypertrophic cardiomyopathy (cardiomegaly), mild left ventricular dysfunction, tracheal tug, possible laryngomalacia and thus the risk of perioperative heart failure with anticipated difficult weaning off ventilatory support in the post operative period. However the procedure was considered essential and the anaesthetic risk justifiable as the pacemaker implantation would allow early recovery and growth of this sick infant. General anaesthesia was administered with controlled ventilation using ketamine, rocuronium and sufentanil. A severe glottic edema was observed on laryngoscopy. A 3.5 mm ID (Portex) polyvinyl uncuffed endotracheal tube was placed orally with utmost difficulty. For patient was safety invasive monitoring was provided and external pacing was kept standby. A 'Medtronic Sigma' pacemaker, Minneapolis, USA[VVIR, rate: 100/min [Figure 1](B) was placed in a subcostal pouch and two epicardial steroid-eluting-button pacing leads were stitched onto the LV lateral wall through a left anterior 6th ICS thoracotomy [Figure 2](B). The child was not extubated and provided pressure-regulated-volume-controlled ventilation electively until the second post-operative day, with intravenous antibiotics, steroids, nebulized bronchodilators and respiratory physiotherapy. On extubation the child's tracheal tug persisted, but reduced gradually over the following two weeks of therapy.
| Discussion|| |
The classic description of Congenital Heart Block in Neonatal Lupus is: a fetus or newborn discovered to have a slow heart rate due to CHB in the absence of causative structural abnormalities, for which laboratory investigation reveals antibodies to SSA/Ro and/or SSB/La in the maternal serum. Although the mother may have SLE, Sjogren's syndrome (SS) or an undifferentiated autoimmune syndrome (UAS), many mothers are entirely asymptomatic. Many cases are discovered in utero, most commonly between 18 and 24 wk of gestation. The incidence of neonatal lupus in an offspring of a mother with anti-SSA/Ro antibodies is ~2%. When an anti-SA/Ro-positive mother has previously given birth to a child with NLE, the risk of CHB in a subsequent pregnancy rises to ~18%. The degree of heart block includes all levels from 1st degree, discovered only incidentally on electrocardiogram, through 3rd degree (complete) heart block with ventricular rates below 50 beats per minute. Mortality (including fetal demise) is ~20% . Our patient, born to a strongly seropositive mother, had a complete CHB and was treated successfully with an urgent pacemaker implantation on presentation at 8 weeks of age. The urgency of the procedure was warranted as the child had mild cardiomegaly, left ventricular dysfunction and possibly early congestive heart failure and onset of cardiomyopathy. Additionally the child had a marked tracheal tug and possible laryngomalacia with an anticipated difficult weaning off ventilatory support. To prevent any further deterioration and allow early recovery the risk and challenge of anaesthesia was considered justified.
As CHB is most often identified between the 18th and 24th week of gestation, intrauterine therapy is possible. The critical decision is whether any treatment is necessary. Guidelines are not well established and are based empirically on anecdotal evidence. The rationale for treatment of identified heart block is to diminish a generalized inflammatory insult and lower the titer of maternal autoantibody. Several intrauterine therapeutic regimens have been tried, including dexamethasone, which is not metabolized by the placenta and is available to the fetus in an active form, and plasmapheresis. Maternal risks of dexamethasone are similar to those of any glucocorticoid and include infection, osteoporosis, osteonecrosis, diabetes, hypertension and preeclampsia. Fetal risks include oligohydramnios, intrauterine growth retardation and adrenal suppression. Fetuses with very slow heart rates and hydrops have been treated with sympathomimetics via the maternal circulation, or even digoxin or fetal pacing, as well as early delivery. In the absence of controlled studies (which may never be feasible given the rarity of CHB), these measures should be considered highly experimental and only reserved for fetuses in life-threatening situations like hydrops and deteriorating cardiac function. None of these prenatal treated modalities were provided to our patient. After birth, treatment of the symptomatic infant often involves pacemaker therapy, and supportive treatment for low output or congestive heart failure. Despite the presence of intact antibody against SSA/Ro and SSB/La in breast milk, breastfeeding appears to confer no risk in the child's disease when compared with formula, although caution should prompt cessation of breastfeeding in a case of worsening cardiomyopathy (CM) or rash. Approximately two-thirds of all recognized cases receive pacemaker insertion before reaching adulthood, and current practice suggests that virtually all patients with complete heart block will have a pacemaker at some point in their lives ,. Risk factors for needing a pacemaker include very slow heart rates (below 55 bpm), symptoms such as poor exercise tolerance, cardiomegaly, long QRS or QT durations, ectopy, syncope, or structural or functional heart disease. Unfortunately, conduction system disease alone is not the whole story in NLE. There is a disturbingly high incidence of late CM leading to low output congestive heart failure, death or transplant, even after successful pacemaker implantation for the associated heart block. Our patient's condition fulfilled the criteria for urgent pacemaker implantation. The child was precious as the mother, who was strongly seropositive for SLE, had two previous abortions. The child had also presented late to this tertiary care hospital inspite of prenatal diagnosis, genetic counseling and advised referral to a higher center at 2 weeks age due to illiterate parents.
With respect to anaesthetic technique no studies have championed one over the other. However, prolongation of QT interval with isoflurane, sevoflurane or desflurane and shortening with halothane has been reported . These inhalational agents, and also propofol, may affect the morphology of sensed intracardiac ECG and induce tachyarrhythmias. Sevoflurane, desflurane, lidocaine and bupivacaine (in high doses) affect defibrillation thresholds. Monitoring with reliable pulse waveform, arterial pressures and best surface lead ECG for P-wave and ischemia diagnosis are recommended. Halothane, isoflurane, sevoflurane, thiopentone and propofol are known to depress the myocardium and decrease systemic vascular resistance. Opioids (e.g. fentanyl), benzodiazepines (e.g. midazolam) and ketamine exert fewer effects on systolic and mean arterial pressures. Thus neonates with complete CHB undergoing general anaesthesia may have risk of cardiac complications. Changes in volume loading, contractility or systemic vascular resistance may predispose to cardiac failure. Patients with symptomatic bradycardia will require chronotropic drugs or backup transcutaneous or transvenous pacing  . Our patient was given general anaesthesia with ketamine, rocuronium and sufentanil with endotracheal intubation and controlled ventilation. Monitoring included pulse oximetry, capnography, ECG, temperature, invasive arterial and venous pressures. An external pacemaker and sympathomimetic drugs were kept standby for cardiovascular emergencies. Ketamine was considered appropriate for our patient due to its favourable cardiovascular effects and also its bronchodilator action keeping the tracheal tug and likely laryngomalacia in mind.
The respiratory distress and gross glottic edema in this case was attributed to laryngomalacia, the most common cause of chronic stridor in children. Laryngomalacia occurs due to an intrinsic defect or delayed maturation of supporting structures of the larynx. The airway is partially obstructed during inspiration by the prolapse of the flaccid epiglottis, arytenoids and aryepiglottic folds. The inspiratory stridor is usually worse when the child is in a supine position, when crying or agitated, or when an upper respiratory tract infection occurs  , as was seen in this case. Our patient was provided controlled ventilation intraoperatively and pressure regulated volume controlled ventilation for two post operative days. On follow-up the child's tracheal tug and stridor gradually improved with oxygen, steroids, bronchodilators, antibiotics and physiotherapy. At hospital discharge after two weeks of supportive therapy the lungs were clear, tracheal tug was minimal and the child was thriving well.
In conclusion, the authors report a rare case of neonatal lupus erythematosus with complete congenital heart block, cardiomegaly, mild left ventricular dysfunction, severe respiratory distress, tracheal tug and a possible laryngomalacia who was given a challenging high risk anaesthesia for urgent permanent pacemaker implantation at 8 weeks of age. There was gross glottic edema making intubation most difficult. A safe anaesthetic technique using ketamine, controlled ventilation, monitoring with invasive arterial pressures and standby emergency external pacemaker was provided to our patient with satisfactory outcome. Post operatively the patient was also provided elective ventilation with supportive therapy and recovered well.
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[Figure 1], [Figure 2]