Year : 2011 | Volume
: 14 | Issue : 2 | Page : 158--161
Bipolar hip arthroplasty in an adult patient with uncorrected tetralogy of fallot: Anesthetic management
Ajmer Singh, Deepa Sarkar, Bhuvnesh Kansara, KK Sharma
Departments of Anaesthesiology and Critical Care, Escorts Heart Institute and Research Centre Ltd., New Delhi, India
Escorts Heart Institute and Research Centre Ltd.,Okhla Road, New Delhi - 110 025
|How to cite this article:|
Singh A, Sarkar D, Kansara B, Sharma K K. Bipolar hip arthroplasty in an adult patient with uncorrected tetralogy of fallot: Anesthetic management.Ann Card Anaesth 2011;14:158-161
|How to cite this URL:|
Singh A, Sarkar D, Kansara B, Sharma K K. Bipolar hip arthroplasty in an adult patient with uncorrected tetralogy of fallot: Anesthetic management. Ann Card Anaesth [serial online] 2011 [cited 2020 Jul 6 ];14:158-161
Available from: http://www.annals.in/text.asp?2011/14/2/158/81577
Congenital heart disease occurs in 0.5-1% of all live births.  Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease, and accounts for 10% of all congenital heart diseases. In modern anesthesia practice, uncorrected TOF lesions are rarely encountered in their adulthood. Nonoperated TOF patients have a survival rate of 30% at 10 years and <3% at 40 years.  The perioperative management of adult patients with uncorrected TOF is a challenge for the anesthesiologists owing to the long-term effects of hypoxia and decreased pulmonary blood flow, resulting in considerable modification of the physiology. We report the successful management of an adult patient with uncorrected TOF who underwent hip arthroplasty.
A 36-year-old man, weighing 60 kg, with fracture of the left hip was transferred from a city hospital to our center for hip arthroplasty. The patient had uncorrected TOF, which was diagnosed and confirmed by transthoracic echocardiography (TTE) in early childhood. Past history included drainage of the large intracerebral abscess at the age of 8 years. Following that surgery, the patient suffered left hemiplegia, which recovered nearly completely.
Preoperative assessment revealed poor exercise tolerance with New York Heart Association class III symptoms. On examination, the patient was cyanosed and had clubbing. Grade IV/VI ejection systolic murmur was heard at left sternal border in the 2 nd -3 rd intercostal space. The heart rate (HR) was 88 beats/min and the blood pressure (BP) was 112/78 mmHg. Saturation (SpO 2 ) while breathing room air was 77%. A 12-lead electrocardiogram showed sinus rhythm, right axis deviation, right ventricular hypertrophy and incomplete right bundle branch block. TTE showed normally related great vessels, two patent atrioventricular valves, persistent left superior vena cava to coronary sinus, absent right superior vena cava, nonrestrictive perimembranous ventricular septal defect with right to left (R→L) shunt, severe pulmonary stenosis with tiny antegrade flow to the branch pulmonary arteries, hypertrophied right ventricle and normal right/left ventricular function. Blood chemistry revealed hematocrit of 57.8%, elevated activated partial thromboplastin time (37.1 sec vs. 26.4 sec control), an international normalized ratio of 1.32 and a platelet count of 271,000/mm 3 . Hepatic and renal parameters were within normal limits.
Left bipolar hip arthroplasty under general anesthesia was planned. While the patient was kept fasting for surgery, normal saline at 1 ml/kg/h was commenced. The operating room temperature was kept at 25°C. During assembly of the intravenous tubings and pressure transducers, care was taken to remove all air bubbles. The patient was premedicated with oral lorazepam 1 mg 45 min prior to scheduled surgery and pulse oximeter was connected on arrival in the operation theater. Intraoperative monitoring included electrocardiogram, SpO 2 , end-tidal carbon dioxide, respiratory gas monitor, temperature, invasive arterial pressure by radial artery cannulation, central venous pressure (CVP), TEE and urine output monitoring. Prior to induction, the arterial blood gas analysis showed the following values: pH 7.40, partial pressure of carbon dioxide 35 mmHg, partial pressure of oxygen 43 mmHg, base deficit -1.7 m mol/L and saturation 77%. Anesthesia was induced with intravenous administration of midazolam 1.5 mg, fentanyl 100 μg, etomidate 12 mg and vecuronium 8 mg. After endotracheal intubation, the left internal jugular vein was cannulated with 5 F double lumen catheter; subsequently transesophageal echocardiographic (TEE) probe was placed. Anesthesia was maintained with oxygen in air (50:50), sevoflurane (end-tidal concentration 2-2.5%) and incremental doses of fentanyl. Antibiotic prophylaxis was achieved with cefoperazone and amikacin. A four-chamber view on TEE with color Doppler confirmed the findings of TTE. A short-axis view at the midpapillary level was used to evaluate the volume status and global ventricular function. Norepinephrine infusion was kept ready to treat any systemic hypotension, but was not required. Intraoperative fluid administration was guided by central venous pressure (CVP) and TEE monitoring. The systolic BP varied from 108 to 136 mmHg, diastolic BP varied from 55 to 80 mmHg, HR varied from 74 to 106 beats/min, CVP varied from 7 to 10 mmHg and SpO 2 varied from 83 to 93%. Surgery was performed in a right lateral position, using a noncemented 13-mm femoral stem and 22/45-mm femoral head (Hastings, De Puy International Ltd. Leeds, United Kingdom). Intraoperative fluid losses and blood loss of 300 ml were replaced with 1500 ml of ringer lactate and 300 ml of fresh frozen plasma. The total urine output was 300 ml. Surgery lasted 72 min. The patient's trachea was extubated and transferred to the intensive care unit. Postoperative analgesia was achieved with intravenous infusions of tramadol (100 mg 8 hourly) and morphine (3 mg 8 hourly). Low-molecular weight heparin (dalteparin, 2500 IU 12 hourly) was administered subcutaneously to reduce the risk of thromboembolism. Postoperative SpO 2 on room air was 78%. After an uneventful recovery, the patient was shifted to the ward on day 1 and discharged from the hospital on day 5.
TOF is comprised of four anomalies: membranous ventricular septal defect, overriding aorta, right ventricular outflow tract (RVOT) stenosis and right ventricular hypertrophy.  The natural history of patients is variable, and more than 95% of the patients die by the age of 40 years. Cyanosis in these patients is caused by an insufficient pulmonary blood flow and mixing of arterial blood by venous blood (R→L shunt). Adverse effects of cyanosis on various organs and systems include ventricular dysfunction, secondary polycythaemia, hyperviscosity, decrease in von Willebrand factor and platelet function, increased fibrinolysis, proliferative lesions in glomeruli and increased incidence of cerebral abscesses.  It is very rare to encounter a 36-year-old patient of uncorrected TOF undergoing major noncardiac surgery. However, sporadic reports of transurethral resection of the bladder in a 74-year-old  and laparotomy in a 39-year-old patient  are mentioned in the literature.
The goals of anesthetic management in patients with TOF are aimed toward: (i) maintenance of normovolaemia, (ii) avoidance of a decrease in systemic vascular resistance (SVR) and (iii) avoidance of an increase in pulmonary vascular resistance (PVR). The "tet spell" or hypercyanotic attacks are generally triggered by a decrease in SVR or a spasm of cardiac muscle in the region of the RVOT, resulting in an increase in the magnitude of the R→L shunt. The appropriate treatment of the test spell depends on the cause of the spell. If the spell is due to a decrease in SVR, it can be treated with an a-adrenergic agonist such as phenylepherine or norepinephrine, and intravenous fluids. If the hypercyanotic attack is due to cardiac muscle spasm, propranolol or esmolol may be used to treat the spell.
Regional anesthesia, particularly spinal block, should be avoided in these patients as it can cause relative hypovolaemia and decrease in SVR. Presence of coexisting coagulopathy precludes the use of epidural block. General anesthesia is the preferred technique as it allows better control of the hemodynamic and respiratory parameters and placement of TEE probe. It is mandatory to keep the patient normovolaemic as hypovolemia increases the R→L shunt because of the low-systemic pressure. In addition, loss of preload also increases the systolic narrowing of the RVOT and deepens the cyanosis. Hyperviscosity increases the risk of spontaneous thromboses, particularly when these patients are fasting or dehydrated. To increase the pulmonary blood flow, factors that increase PVR should be avoided, i.e. hypoxia, hypercarbia, hypothermia, acidosis, increased sympathetic tone and use of sympathomimetic drugs (dopamine, epinephrine). Sympathomimetics could also increase the cardiac muscle spasm and worsen hypoxemia. b-blockers (propranolol, esmolol) are helpful to relieve the systolic narrowing of the RVOT in case of dynamic obstruction, whereas a-stimulants (norepinephrine, phenylephrine) decrease the shunted component of the right ventricular stroke volume and increase the flow through the pulmonary tree and thus help in fixed obstruction situations.
SpO 2 is an effective and immediate means of monitoring the degree of shunting in patients with congenital heart disease as it gives information on the effective pulmonary blood flow, ratio of systemic/pulmonary blood flow and variations of PVR and SVR. When hypotension is associated with a decrease in the SpO 2 , an increase in the R→L shunt is the probable cause. In such a situation, we had planned to administer norepinephrine infusion. However, this did not become necessary. In the presence of a R→L shunt, end-tidal CO 2 underestimates the PaCO 2 because of the deadspace effect of the blood volume avoiding the lungs.  An invasive BP monitoring gives instantaneous data on the volume status, SVR and systemic function and allows frequent arterial blood gas analysis.  CVP monitoring is helpful to guide intaoperative and postoperative fluid therapy and infusion of vasoactive drugs. The CVP catheter must be taken out at the earliest because of the increased risk of thrombosis in such patients. TEE allows real-time assessment of cardiac filling, ventricular and valve function, regional wall motions and the magnitude of R→L shunting and, therefore, allows recognition of the possible etiology of any hemodynamic instability. Maintaining normothermia is mandatory as hypothermia may induce pulmonary vasoconstriction and coagulation disorders. Hypothermia is considered as an independent predictor of postoperative cardiac complications. 
The choice of the anesthetic agents is governed by their hemodynamic consequences. The circulatory effects of all intravenous drugs are well known: the rate and dose are more important than the actual drug used. Infusion of air bubbles into the vascular system can result in air embolism to the coronary or cerebral arteries; hence, air filters should be placed in all intravenous lines. Sedative premedication is helpful to reduce myocardial oxygen demand, but care must be taken in cyanotics as the SpO 2 level may drop below the acceptable limits. The most cardiostable induction agent is etomidate.  Its beneficial effects include rapid onset of action, rapid recovery and maintenance of cardiovascular stability in both healthy and hemodynamically compromised patients. Ketamine can also be used as an induction agent owing to its central sympathetic stimulation properties, but there is a theoretical risk of worsening of the infundibular spasm produced by increased myocardial contractility. At lower concentrations, the administration of volatile anesthetics improves arterial oxygenation by causing relaxation of the muscle spasm in RVOT, and decreases the total body oxygen consumption. 
Intraoperative blood loss should be replaced with an adequate amount of intravenous fluids and fresh frozen plasma. A noncemented prosthesis was used to reduce the risk of systemic hypotension and pulmonary hypertension associated with cemented hip arthroplasty. Murphy et al. found a significant decrease in the systemic pressure, associated with reduction in stroke volume and increase in PVR, within 1 min of administration of cemented prosthesis. Early extubation is recommended to reduce the risk of increase in PVR.  Patients with congenital heart disease are at an increased risk of endocarditis and should benefit from an appropriate antibiotic. Adequate pain control is essential to avoid pain-induced increase in sympathetic tone. Early mobilization and use of low-molecular weight heparin is helpful in reducing the incidence of thromboembolism.  Postoperative care of these patients should preferably be provided in a high-dependency unit with facility for invasive monitoring and early intervention. 
In conclusion, noncardiac surgery in an adult patient with uncorrected TOF is best performed in a dedicated tertiary center that has experience in the treatment of such patients. A multidisciplinary approach is essential for knowledge of the underlying pathology, assessment of the degree of cardiovascular impairment and careful intraoperative and postoperative management plan.
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