THE PATIENT WITH PULMONARY HYPERTENSION

ANAESTHESIA FOR THE PATIENT WITH
PULMONARY HYPERTENSION
ANAESTHESIA TUTORIAL OF THE WEEK 228

JUNE 2011
Dr Sarah Thomas, Senior Anaesthetic Registrar
Royal Hobart Hospital
Correspondence to sarah.thomas@dhhs.tas.gov.au
QUESTIONS
Before continuing, consider the following scenario and question.  The answers can be found at the end
of the article, together with an explanation.
You are to anaesthetise a 65-year-old woman for laparoscopic sigmoid colectomy.  She has recently
been diagnosed with colorectal carcinoma.  The patient has been a heavy smoker in the past and has
severe chronic obstructive pulmonary disease (COPD), with secondary pulmonary hypertension.  She
also has essential hypertension.  Medications include a beta-blocker, ACE inhibitor, inhaled
steroid/beta-agonist and aspirin.
What are your concerns in anaesthetising this patient?
INTRODUCTION
The disease spectrum of Pulmonary Hypertension (PH) has received greater interest in the past decade,
as specific therapies have been developed and survival has improved.  More patients with PH are now
presenting for surgery, and this poses a challenge to the anaesthetist.  Knowledge of the underlying
physiology is paramount in preventing the feared complication of right heart failure.
DEFINITION AND CLASSIFICATION
Pulmonary Hypertension is defined as a mean pulmonary artery pressure (PAP) >25mmHg at rest with
a pulmonary capillary wedge pressure <12mmHg.   Pulmonary hypertension is considered moderately
severe when mean  PAP >35mmHg.  Right ventricular failure is unusual unless mean PAP is
>50mmHg.
The World Health Organisation classifies pulmonary hypertension by aetiology into five groups. The
disease, including its classification, was comprehensively reviewed at the 4
th World Symposium on
Pulmonary Hypertension in 2008.
Table 1: Clinical classification of pulmonary hypertension
1 Pulmonary Hypertension (PAH)
2 Pulmonary hypertension owing to left heart disease
3 Pulmonary Hypertension owing to lung disease
4 Chronic thromboembolic pulmonary hypertension (CTEPH)
5 Pulmonary hypertension with unclear multifactorial mechanisms
Group 1 includes the disease idiopathic pulmonary hypertension (formerly known as primary
pulmonary hypertension), as well as PH associated with connective tissue disorders. This group of
diseases share similar pathological findings and clinical appearance.  The incidence of idiopathic PH is
higher than previously thought, although remains relatively rare at 15 per million.
Of greater interest to the anaesthetist are the more common forms of PH: those due to left heart disease
(group 2) and those due to lung disease (group 3).  Cardiac anaesthetists have long been familiar with
PH due to left heart disease,  which often occurs  in patients undergoing cardiac surgery.  Examples
would include patients with mitral valve disease undergoing valve replacement, or patients with severe
LV failure undergoing coronary bypass surgery.
Non-cardiac anaesthetists are more likely to encounter PH in patients with lung disease.  Underlying
diseases include COAD, interstitial lung disease, and sleep disordered breathing.  The majority of
patients in this group have modest PH.
PITFALLS IN DIAGNOSIS
Pulmonary hypertension may be suspected after  patient  assessment  based on history, examination,
ECG and chest x-ray.  The symptoms of PH are non-specific, and diagnosis can be delayed.
If PH is suspected, transoesophageal echocardiography (TTE) is usually the first investigation
undertaken.  TTE utilizes Doppler across a tricuspid regurgitant jet, to estimate pulmonary artery
pressure.  This technique has been shown to under or over estimate PAP in up to half of patients at risk
of PH, and therefore as a diagnostic test has limitations in accuracy.
Right heart catheterization is required to confirm the diagnosis.  A vasodilator challenge forms part of
this assessment.

Neuroleptic Malignant Syndrome

The Indian Anaesthetists’ Forum – (http://www.theiaforum.org)                    Online ISSN 0973-0311
January 2010(4)
Agrawal P,Agrawal A, Singh I: Neuroleptic Malignant Syndrome and Anaesthesia : A Case Report  1
Neuroleptic Malignant Syndrome and Anaesthesia: A Case Report
1. DNB Resident Presently working as Senior Resident, JPNATC, AIIMS, New Delhi
2. Consultant, 3. Head of the Department
Department of Anaesthesiology and Intensive Care
Jaipur Golden Hospital, Rohini, New Delhi.
Correspondence: Pramendra Agrawal (pramendraagrawal@yahoo.com)  
About the Author: Dr Pramendra Agrawal passed
DNB in Anaesthesiology in the year 2008. He is
presently working as Senior Resident in Jai Prakash
Narayan Apex Trauma Centre, All India Institute of
Medical Sciences [AIIMS], New Delhi, India. His field
of interests include trauma care, regional interventional techniques and intensive care.
Neuroleptic Malignant Syndrome (NMS) is a life threatening, neurological disorder most
often caused by an adverse reaction to neuroleptic or anti psychotic drugs. We report a case
of Neuroleptic Malignant Syndrome who was posted for an incidental surgery and its
anaesthetic management.
Key Words: Neuroleptic Malignant Syndrome, anti psychotics, hyperthermia
Neuroleptic Malignant Syndrome (NMS) is a rare but  potentially life threatening
idiosyncratic reaction to neuroleptic drugs. It causes hyperthermia, muscular rigidity, altered
mental status, elevated creatine phosphokinase (CPK) and autonomic dysfunction. The
underlying pathological abnormality is thought to be the central dopamine D2 receptor
blockade or dopamine depletion in the hypothalamus, nigrostriatal and spinal pathways.
The condition shares many features with the serotonin syndrome and malignant
hyperthermia. Anaesthesia for an incidental surgery in such a patient poses unique
challenges to an anaesthesiologist.  
Case Report: An 18 year old male, a known case of Bipolar mood disorder on antipsychotics
was admitted to our Intensive Care Unit with complaints of high grade fever (106F) for 2
days, agitation and involuntary movements for 8 days and vomiting with altered sensorium
for 1 day. Patient was immediately intubated to protect his airway, intensive measures to
bring down temperature commenced and investigations including haematological, biochemical, brain imaging and cerebrospinal fluid sent for evaluation. Investigations revealed:
 elevated liver enzymes (SGOT 477 IU/l, SGPT 190  IU/l);
progressively increasing Creatine Kinase (1004; 2040; 3270; 39794 U/l), Serum Creatinine
(1.3; 1.5; 1.8 mg/dl) and Serum K+ (4.8, 4.9, 5.5 meq/l). Computed tomography of brain and
cerebrospinal fluid examination were normal.  So a  diagnosis of neuroleptic malignant
syndrome was made.  All antipsychotic medications were then stopped.  Tab. Bromocriptine
1.25 mg thrice daily and Tab. Alprazolam 0.25 mg four times daily were started and other
intensive care measures continued. Patient was gradually weaned off ventilator support and
extubated on 6th day.
Unfortunately patient developed a bed sore on the buttock which needed a flap
cover He was posted for surgery on 15th day of admission. A pre-anaesthetic check up
revealed a responsive patient, hypertonia present in all limbs and restricted mouth opening
(just 2 fingers due to hypertonia). Investigations  revealed elevated CPK 829 U/l, S. K+ 4.9
meq/l, INR = 1.54; rest of examination and investigations were within acceptable limits.
Anaesthetic management included avoidance of following drugs perioperatively: Inj.
droperidol, succinylcholine, prochlorperazine, promethazine and metoclopramide. Patient
received premedication with alprazolam 0.25 mg at 10 p.m. before the day of operation and
at 6 a.m. on day of surgery. Consent taken and patient shifted to operation theatre. Drip
started with 16 G intravenous cannula and standard  monitoring established. Fentanyl 1.5
µg/kg and midazolam 1 mg IV was administered. Anaesthesia induced by thiopentone
sodium 4 mg/kg IV slowly, after pre-oxygenation or  5 minutes and adequacy of mask
ventilation confirmed, muscle paralysis achieved with atracurium 0.5mg/kg. Airway secured
with 34Fr cuffed armoured endotracheal tube orally and anaesthesia maintained with 50%
of O2 + N2O + Isoflurane < 0.4%, fentaynl was used for analgesia and atracurium for muscle
paralysis assisted by neuromuscular monitoring. Surgery was conducted in prone position;
procedure lasted 2½   hours during which 2 units of whole blood, 2 units of FFP and 1.5 litres
crystalloid were infused. Patient remained haemodynamically stable throughout procedure.
At end of surgery, neuromuscular blockade was reversed with neostigmine and
glycopyrrolate. Trachea was extubated in prone posture itself as requested by surgeons to
avoid pressure on flap after patient was awake; Ondansteron 4mg IV was used as
antiemetic.
Post operatively patient was shifted to post anaesthesia care unit with O2
supplementation and then towards after 2 hrs. Patient was discharged from hospital on 7th
post operative day with psychiatry referral.
Discussion: Neuroleptic Malignant Syndrome (NMS) was first described by Delay et al during
early trials of haloperidol. The incidence is estimated to range from 0.02–2.4% with The
Diagnostic criteria are:
• Administration of neuroleptics
• Hyperthermia (> 38oC)
• Muscle rigidity
• Five of following: mental status change, tremor, tachycardia, incontinence, labile
blood pressure, metabolic acidosis, tachypnoea/hypoxia, CPK elevation,
diaphoresis/sialorrhea, leukocytosis.
• Exclusion of other central and systemic causes of hyperthermia.
 Although NMS has a variable onset and sometimes evolves rapidly, rigidity and
altered mental status usually occur early, followed by autonomic changes and
hyperthermia. No laboratory tests are pathognomonic of diagnosis. Serum creatine kinase
is frequently elevated reflecting rhabdomyolysis, with resultant risk of myoglobinuric renal
failure. CT scan brain and cerebrospinal fluid examination and sepsis evaluation are negative
in NMS and allow for   the exclusion of other causes of fever and neurological deterioration.
Other frequently described laboratory abnormalities include metabolic acidosis, hypoxia,
low serum iron, electrolyte abnormalities, elevated serum catecholamines and
coagulopathies
Differential Diagnosis of NMS: Infectious encephalitis4,5, structural lesion of brain, rare
cases of status epilepticus6, lethal catatonia7, heat stroke, endocrinopathies, drugs,
autoimmune disorders, thyrotoxicosis, phaeochromocytoma, malignant hyperthermia,
serotonin syndrome. Volatile anaesthetics and succinylcholine are associated with
malignant hypertherma during surgery, which can be confused with NMS if neuroleptics are
administered

The basic management of NMS remains risk reduction, early diagnosis, cessation of
neuroleptic medications and institution of Intensive, medical and nursing care.
Benzodiazepines, bromocriptine, amantadine or other dopamine agonists may be a
reasonable next step in patients with moderate symptoms of NMS. Dantrolene may be
beneficial in cases of NMS with extreme rigidity and hyperthermia. Electro convulsion
therapy (ECT) is used if NMS is refractory to other measures or who remain psychotic after
NMS   is resolved. Since a common pathophysiology has been suggested between NMS and
malignant hypertherima (MH) the possibility that patients with a history of NMS may be
vulnerable to developing MH is an important factor when considering general anaesthesia,
especially succinylcholine administration. To date, there is no report in the literature of
(MH) as a complication of ECT in NMS patients. However, until the association between
NMS and MH is conclusively disproved, careful metabolic monitoring of general anaesthesia
is necessary.
Conclusion: Neuroleptics are highly effective medications that have achieved wide spread
use in medicine and psychiatry. However they have been associated with NMS in about 0.2
percent of patients. Awareness of diagnosis, cessation of medications, early medical
intervention and consideration of specific remedies can reduce morbidity and mortality
when NMS occurs. This case report has been published to increase familiarity with the
diagnosis and management of this unusual but fascinating drug reaction and anaesthetic
management of a incidental surgery in a patient of NMS.
References:  
1. Delay J., Pichot P., Lemperiere T, Elissade B., Peigne F. Un neuroteptique majeur non
phenothiazine et non reserpinique, L’haloperidol dans Le traitment des psychoses.
Annales Medico-Psychologique 1960 ; 118:145 – 152.
2. Ananth J Parameswaran S., Gunatilake S. et. al.; Neuroleptic malignant syndrome
and a typical antipsychotic drugs. J. Clin Psychitary 2004 Apr: 65 (4): 464- 70.  
3. Velamoor VR, Normal RM, Caroff SN, Mann SC, Sullivan KA, Antelo RE. Progression of
symptoms in Neuroleptic Maligant Syndrome. Journal  of Nervous and Mental
Disease : 1994 ; 182: 168 – 173.
4. Caroff SN, Mann SC. McCarthy M, Naser J., Rynn M, Morrison M. Acute infectious
encephalitis complicated by Neuroleptic Malignant Syndrome. Journal of Clinical
Psychopharmacology 1998 ; 18 : 349-351
5. Caroff SN, Mann SC, Gliatto MF, Sullivan KA, Campbell EC. Psychiatric Manifestations
of acute viral encephalitis. Psychiatric Annals 2001: 31; 193-204.
6. Caroff SN, Mann SC, Neuroleptic Malignant Syndrome. Medical clinics of North
America 1993; 77: 185-202
7. Mann SC, Caroff SN, Bleier HR, Welz WKR, Kling MA, Hayashida M. Lethal catatonia ;
1986; 143: 1374 – 1381
8. Keck PE Jr. Arnold LM. The serotonin syndrome. Psychiatric Annals 2000 : 30 : 333 –
343
9. Caroff SN, Rosenberg H., Mann, Campbell EC, Gliatto MF, Sullivan KA, Neuroleptic
Malignant Syndrome in the perioperative setting. American Journal of
Anesthesiology 2001,: 28 ; 387 – 393
10. Davis JM, Caroff SN, Mann SC. Treatment of neuroleptic malignant syndrome
Psychiatric Annals 2000; 30: 325 – 331.  
11. Caroff SN, Mann SC, Keck PE, Jr. Specific treatment of the neuroleptic malignant
syndrome. Biological Psychiatry 1998; 44: 378 – 381.   The Indian Anaesthetists’ Forum – (http://www.theiaforum.org)                    Online ISSN 0973-0311
January 2010(4)
Agrawal P,Agrawal A, Singh I: Neuroleptic Malignant Syndrome and Anaesthesia : A Case Report  5
12. Denborough MA, Collins SP, Hopkinson KC. Rhabdomyolysis and malignant
hyperpyrexia. Br Med J 1985; ii: 1878
13. Tollefeson G. A case of neuroleptic malignant syndrome in vitro muscle comparison
with malignant hyperthermia J. Clin Psychopharmacol 1982; 2: 266 – 70  

pediatric trauma

Management principles of pediatric trauma patients

Management principles of pediatric trauma patients are similar to those of adults, but modified according to the age group of the child. Children are not just small adults. Their unique, developing psychologic, anatomic, and physiologic characteristics pose special challenges to anesthesiologists and the entire trauma care team. Optimal management of the pediatric trauma patient depends on adequate knowledge and

understanding of these unique characteristics.

INITIAL ASSESSMENT AND MANAGEMENT

Primary Survey

The main goal of the primary survey is to rapidly find all potentially life-threatening injuries to prioritize management for efficient resuscitation and achieve hemodynamic stability. This requires immediate assessment of the “ABCDEs” of the Advanced Trauma Life Support (ATLS) protocol and constant reevaluation of the adequacy of resuscitation strategies.

Airway with C-Spine Control

Evaluation of the airway in an injured child can be complex. Injury to the airway or nearby structures may distort normal anatomy and render mask ventilation and tracheal intubation difficult.Preexisting conditions thatmay complicate emergency

airway management include congenital abnormalities, such as micrognathia (mandibular hypoplasia), macroglossia, and cleft palate and the presence of obstructive sleep apnea with or without obesity.

Inspection of the airway includes the face, mouth, mandible, nose, and neck. Look for edema, foreign bodies, secretions, blood, loose or missing teeth, and fractures of the jaw, mandible, and cervical spine. Any trauma victim, especially one with a closed-head injury, is presumed, until proved otherwise, to have cervical spine (C-spine) injury and a full stomach.

C-spine precautions

should be maintained and techniques that minimize the risk of pulmonary aspiration should be taken at all times.

Healthy neonates and young infants have large heads, including prominent occiputs relative to body size, so that, in the supine position, the infant neck is naturally flexed on the chest and the supine infant headmay be flexed on the neck]. This has several important implications.

The natural head and neck flexion of the obtunded or sedated young infant often results in significant airway obstruction that may be relieved by gently lifting the chin up and forward (anteriorly) to slightly extend the head on the neck. Otherwise, an

oral airway can be inserted with no relative movement of head and neck. In suspected C-spine injury, a more neutral, straight head and neck position should be achieved by placing a blanket or pad under the supine infant or young child’s torso.

Neonatesandyounginfants areobligate nose breathers until three to five months of age so that any secretions or blood in their relatively narrow nasal passages can lead to airway obstruction.

The larynx in infantsandchildren ismore cephalad, approximately at the level of the C3–C4 vertebrae in infants compared with the C5–C6 level in adults. This may give the impression that the infant larynx is more anterior during direct laryngoscopy.

The length of the trachea is only 4–5 cm in infants and approximately 7 cm by 18 months of age, so right mainstem intubation or ETT dislodgement can occur with correspondingly small movements of the infant’s head. extubation.When choosing the appropriately sized ETT, keep in mind that in children less than 5 years old, the narrowest part of the upper airway is at the level of the cricoid cartilage, not at the glottis, as in adults. The size of the ETT appropriate for the patient’s age may

be estimated by comparing the tube size with that of the infant or child’s fifth finger, or by using the formula: ETT tube size (diameter in mm) = 4 + (1/4) age .

An air leak around the ETT at 15–20 cmH2Opressure and easy passage of the tube into the trachea clinically suggests that the ETT size is appropriate. The following formula may be used as a guide to determine the appropriate depth of the ETT placement (in centimeters from lips to tip of ETT) for children older than 2 years: 13 + (1/2) age); for under 1 year old: 8 + weight in kilograms [20].

The appropriate depth of ETT insertion may also be approximated bymultiplying the internal diameter (millimeters) of the ETT by 3.

Indications for Endotracheal Intubation

a. Loss of consciousness or altered level of consciousness with inability to protect the airway

b. Inability to maintain patency of airway or clear secretions

c. Provide positive pressure ventilation and adequate oxygenation

d. Significant burn with airway injury.

Different Methods Of Intubation In Difficult Situations