Table of Contents  
Year : 2016  |  Volume : 9  |  Issue : 6  |  Page : 768-770  

A case of uncorrected adult tetralogy of Fallot for emergency decompressive craniotomy: An anesthetic challenge!!

Department of Anaesthesiology, LTMMC and LTMGH, Sion, Mumbai, Maharashtra, India

Date of Web Publication16-Nov-2016

Correspondence Address:
Amruta Vinod Hippalgaonkar
Plot No. 707, Sahyadri Nagar, N-5, CIDCO, Aurangabad - 431 003. Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-2870.194230

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Cyanotic heart disease (CHD) includes those anatomical heart defects that produce a limitation in pulmonary blood flow or result in mixing of oxygenated and deoxygenated blood. Both conditions lead to decreased blood oxygen content and cyanosis. The most frequent defects seen in the outpatient adult congenital setting include tetralogy of Fallot (TOF). The cardiovascular anatomy and physiology of adult CHD is complex and requires specific knowledge of the defect and its anesthetic implications. Hence, they should receive care with multidisciplinary collaboration among anesthesiologists, cardiologists, surgeons, and intensivists. We hereby report the anesthetic management of such a case of head injury in an adult uncorrected TOF with a good outcome.

Keywords: Adult uncorrected tetralogy of Fallot, anesthetic management, head injury

How to cite this article:
Hippalgaonkar AV, Yadav SS. A case of uncorrected adult tetralogy of Fallot for emergency decompressive craniotomy: An anesthetic challenge!!. Med J DY Patil Univ 2016;9:768-70

How to cite this URL:
Hippalgaonkar AV, Yadav SS. A case of uncorrected adult tetralogy of Fallot for emergency decompressive craniotomy: An anesthetic challenge!!. Med J DY Patil Univ [serial online] 2016 [cited 2020 Aug 4];9:768-70. Available from:

  Introduction Top

Tetralogy of Fallot (TOF) is the most common form of cyanotic heart disease (CHD) and accounts for 10% of all cases of congenital heart disease. [1] The perioperative risk is substantially increased in adult uncorrected patients with head injury particularly in those with poor functional class, pulmonary hypertension, congestive heart failure, and cyanosis. The anesthesiologist should be familiar with the patient's specific anatomy and physiology to anticipate intraoperative events that may precipitate acute changes in the magnitude or direction of intracardiac shunts or modulate flow through systemic to pulmonary shunts. [2] So also, attention needs to be paid to neuroprotection.

  Case Report Top

A 28-year-old male patient, weighing 50 kg, bookseller by occupation hailing from Boisar (Mumbai), presented to the emergency department with a history of head injury due to trivial fall at home on the same day associated with loss of consciousness for 15 min followed by drowsiness and one episode of projectile vomiting. There was no history suggestive of fever, any drug, or alcohol intake. There was no history of convulsion, blunt chest trauma, abdominal trauma, ENT bleed, or spine injury.

Relatives gave a history of patient having some congenital heart disease diagnosed in childhood with no details available. The patient was not on any medication for the same. The patient had no other significant medical problems and family history was not contributory.

On examination, the patient had altered sensorium with stable vital signs (heart rate [HR] of 70/min and blood pressure [BP] 118/78 mmHg). His Glasgow Coma Scale was 11/15 (E3M5V3) with B/L pupils constricted and sluggishly reacting to light. Clubbing of fingers was present. The examination of the cardiovascular system revealed parasternal heave with a loud pansystolic murmur and accompanying thrill in the suprasternal region. The other systemic examination was normal.

Computed tomography revealed an acute subdural hematoma of about 2-cm thickness along the right frontoparietal convexity with resultant midline shift of 12 mm to left side with descending herniation causing mass effect on midbrain. Along with linear undisplaced fracture of right temporoparietal bone. A screening echocardiography revealed a large malaligned ventricular septal defect (VSD) of 20 mm with a left-to-right shunt and 50% aortic override with an ejection fraction of 65%. Furthermore, there was evidence of atretic pulmonary valve and peak right ventricular outflow tract (RVOT) gradient of 120 mmHg suggestive of right ventricular pressure overload. Electrocardiogram (ECG) showed right axis deviation with right ventricular hypertrophy. X-ray chest was suggestive of right ventricular hypertrophy with oligemic lung fields. Biochemical investigations were normal except for raised hematocrit (packed cell volume [PCV] 48%). Coagulation profile was within normal limits.

In view of the urgent nature of the surgery, the patient was scheduled for emergency decompression craniotomy. General anesthesia with endotracheal intubation was planned with neuroanesthesia protocols. Preoperative arterial blood gas analysis revealed desaturation with oxygen saturation (SaO 2 ) of 92% and PaO 2 of 55 mmHg. The case was accepted for surgery under the American Society of Anesthesiologists physical status V and consent was obtained.

Inside the operation theater, an intravenous (IV) line was established with 18-gauge. Monitoring included pulse oximetry, ECG, noninvasive blood pressure, end-tidal carbon dioxide, and urine output. A prophylactic dose of antibiotic, ceftriaxone 1 g, was given to provide prophylaxis against infective endocarditis. The patient was given antisialagogue injection glycopyrrolate 0.2 mg IV, and after preoxygenation for 3 min, anesthesia was induced with injection fentanyl 200 μg + injection thiopentone 100 mg (in graded doses) followed by injection vecuronium 5 mg as a muscle relaxant to facilitate endotracheal intubation. Anesthesia was maintained with air + oxygen (50:50), vecuronium bromide 0.1 mg/kg body weight, and sevoflurane (0.4-0.6%). Ventilation was controlled using a closed circuit to maintain slight hypocarbia. Central venous pressure (CVP) monitoring was done through a triple lumen CVP catheter inserted into the internal jugular vein. The bladder was catheterized to monitor the urine output.

Intraoperatively, hemodynamics were HR - 74 beats/min, BP - 106/68 mmHg, and SpO 2 -96-97%. Neuroprotection measures in the form of injection mannitol (1 g/kg) IV 8 hourly, hyperventilation (to a PaCO 2 of 32 mmHg), and seizure prophylaxis (injection eptoin 100 mg) were initiated, to lower the intracranial pressure and make brain lax for the surgeon. Right frontoparietal craniotomy was performed by the neurosurgeon and hematoma was drained. The surgery lasted for about 2 h, and throughout the procedure, the patient remained stable with SpO 2 ranging between 96% and 98% and PaCO 2 between 30 and 33 mmHg. Normal saline was used as maintenance fluid during surgery and about 500 ml of blood loss was replaced with IV fluids. At the end of the surgery, the residual neuromuscular block was antagonized with neostigmine 2.5 mg and glycopyrrolate 0.4 mg followed by extubation, once the patient was awake and responded to verbal commands. Postoperatively, the patient was transferred to the surgical intensive care unit for overnight observation and after 24 h back to the neurosurgery ward with SpO 2 of 90-92%.

  Discussion Top

As described by the French Physician Etienne-Louis Arthur Fallot in 1888, TOF consists of four anatomical components: (1) VSD, (2) an abnormally positioned aortic valve above (overrides) the ventricular septum, (3) RVOT obstruction, and (4) right ventricular myocardial hypertrophy. [3] The reason for presentation with uncorrected lesions is patients with balanced pulmonary and systemic circulations as commonly occurs in patients with complex lesions and may remain relatively asymptomatic until the balance between pulmonary and systemic circulations is disturbed. However, the uncorrected group presents a severe challenge, in issues related to long-term effects of chronic hypoxia. [2] These patients develop severe clubbing and learn to "squat" to increase pulmonary blood flow. They may be polycythemic and suffer complications of long-standing polycythemia: Coagulopathy, intracranial abscess, stroke, hyperuricemia, and neurodevelopmental delay. [3] They have reduced exercise tolerance and are vulnerable to ventricular arrhythmias or sudden death secondary to chronic pulmonary regurgitation, problems related to abnormalities of RV physiology. [3],[4]

In unrepaired TOF patient, pathophysiology depends on the degree of RVOT obstruction. The amount of shunting is determined by the ratio of systemic vascular resistance (SVR) to pulmonary vascular resistance (PVR). With significant RVOT obstruction, infundibular spasm constricts RVOT, limiting blood flow into the pulmonary artery, forcing blood right to left across VSD resulting in significant arterial desaturation or "tet spells." These spells can also be incited by catecholamine release due to pain, stress, and light anesthesia as most of the patients have a dynamic right ventricular outflow obstruction. [2],[5] A hypercyanotic spell presents with rapidly falling saturation. ECG changes suggestive of ischemia may occur and the BP may fall. [3] The treatment involves intravascular volume administration to increase RV stroke volume, increasing depth of anesthesia, avoidance of exogenous catecholamines, increasing FiO 2 , and increasing SVR (with injection phenylephrine) to force blood left-to-right across the VSD and out the RVOT, increasing pulmonary blood flow and thus oxygenation. [6]

An anesthesiologist's concern for a case of TOF with head injury includes: [5]

  • Hypoxia
  • Coagulopathies
  • Lax brain.
Hypoxia leads to hyperviscosity and coagulation abnormality. A hematocrit above 65% requires phlebotomy. If coagulation abnormalities are present, nonsteroidal anti-inflammatory drugs should be avoided, and platelet concentrates should be available perioperatively. [5] In our patient, the PCV was 48% and did not have deranged coagulation profile.

The goal of anesthetic management is to avoid low SVR so as to maintain pulmonary blood flow. Minimize noxious stimulus and avoid catecholamine release in perioperative phase by providing adequate analgesia and anesthesia. Both IV and inhalational drug regimens have been used successfully for induction of anesthesia in TOF to maintain systemic oxygenation and hemodynamic variables. The optimal induction regimen for general anesthesia in TOF should aim to improve arterial blood SpO 2 and to maintain cardiovascular stability. [2] We used a combination of fentanyl and thiopentone in our case for induction of anesthesia which actually improved arterial oxygenation and maintained it between 95% and 98% intraoperatively. Monitoring in the form of pulse oximetry is uniquely important as decreases in arterial saturation can signify increases in PVR and increase in right-to-left shunting. We have to avoid tachycardia and hypovolemia as infundibular stenosis is increased by increasing contractility and HR. Ventilation with high airway pressures can compromise venous return, increase PVR, and exacerbate right-to-left shunting in patients with CHD.

Lax brain is essential from the surgical point of view. A careful use of diuretic minimizes hypovolemia and maintains right ventricular output. Mannitol helps decrease intracranial pressure and cerebral edema. It is ideal as it reduces blood viscosity too. [5] However, one has to be very vigilant as it can cause severe dehydration, hypotension, tachycardia, acidosis, and precipitate "tet spell." Accordingly, we employed hypocarbia and diuretic therapy and avoided hypovolemia by the maintenance of intravascular volume.

  Conclusion Top

The number of adult patients with CHD is rapidly increasing, and presenting with greater frequency for noncardiac surgery. An aggressive multidisciplinary perioperative management resulted in a good outcome of this patient.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Hamid M, Khan MA, Akhtar MI, Hameedullah, Saleemullah, Samad K, et al. Grown up congenital heart disease patient presenting for non cardiac surgery: Anaesthetic implications. J Pak Med Assoc 2010;60:955-9.  Back to cited text no. 1
Cannesson M, Earing MG, Collange V, Kersten JR. Anesthesia for noncardiac surgery in adults with congenital heart disease. Anesthesiology 2009;111:432-40.  Back to cited text no. 2
Hepburn L. Tetralogy of Fallot Anaesthesia Tutorial of the Week 219; 18 th April, 2011. Available from: [Last accessed on 2016 May 04].  Back to cited text no. 3
Lovell AT. Anaesthetic implications of grown-up congenital heart disease. Br J Anaesth 2004;93:129-39.  Back to cited text no. 4
Naqash I, Ahad B, Zargar J, Kirmani A, Wani M. Anaesthetic management of a case of tetrology of fallot for drainage of brain abscess: A case report. Internet J Anesthesiol 2002;7:1-4.  Back to cited text no. 5
Andropoulos DB. Anesthesia for the patient with congenital heart disease for noncardiac surgery. Anesthesiology 2011;206:1-8.  Back to cited text no. 6


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