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Year : 2007  |  Volume : 51  |  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

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 Acceptance11-Mar-2007
Date of Web Publication20-Mar-2010

Correspondence Address:
Usha Kiran
Professor & Head, Department of Cardiac Anaesthesiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110029
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Source of Support: None, Conflict of Interest: None

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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 2020 Jul 3];51:240-3. Available from:

   Introduction Top

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[1]. 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[2] .

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 Top

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 Top

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% [2]. 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[2]. 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[2]. 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 [2],[3]. 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[5]. 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[3]. 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[4] . 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[5]. 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 [6] . 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 [7] , 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.

   References Top

1.Klein-Gitelman MS, Miller ML. Systemic lupus erythemato­sus. In: Behrman RE, Kliegman RM, Jenson HB eds. Nelson Text book of Pediatrics, Philadelphia, 17 th edn 2004; 813-814.  Back to cited text no. 1
2.Buyon JP. Congenital heart block in neonatal lupus: the clinical perspective. Reumatologia 2004; 20:192-197.  Back to cited text no. 2
3.Eronen Marianne, Siren Marja-Kaisa, Ekblad H, Tikanoja T, Julkunen H and Paavilainen T. Short- and long-term outcome of children with congenital. complete heart block diagnosed in utero or as a newborn. Pediatrics 2000;106;86-91.   Back to cited text no. 3
4.Rozner MA, Trankina M. Cardiac pacing and defibrillation. In: Kaplan JA, Reich DL, Lake CL, Konstadt SN eds. Kaplan's Cardiac Anesthesia, Elsevier Saunders, 5th edn 2006;830-831.   Back to cited text no. 4
5.Atlee JL. Arrhythmia, rhythm management devices and catheter and surgical ablation. In: Hensley Jr. FA, Martin DE, Gravlee GP eds. A .Practical Approach to Cardiac Anesthesia, Lippincott, Williams& Wilkins, 3rd edn 2003.; 449-473.  Back to cited text no. 5
6.Ammann J, Winter J, Sunderdiek U, Loer SA. Anesthetic man­agement of neonates with congenital complete heart block un­dergoing pacemaker implantation. Journal of Cardiothoracic and Vascular Anesthesia 2005;19: 212-216.  Back to cited text no. 6
7.Simon NP. Evaluation and management of stridor in the new­born. Clin Pediatr 1991; 30:211-6.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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