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Revista Brasileira de Anestesiologia

Print version ISSN 0034-7094On-line version ISSN 1806-907X

Rev. Bras. Anestesiol. vol.57 no.1 Campinas Jan./Feb. 2007 



Anesthesia for pacemaker implant in an adult patient with unoperated univentricular heart. Case report*


Anestesia para implante de marcapaso en paciente adulto con ventrículo único no operado. Relato de caso



Adriano Bechara de Souza HobaikaI; André Luís Pontes ProcópioI; Marcelo Luiz Souza PereiraII; Aristóteles Pereira CoimbraI; Magda Lourenço Fernandes, TSAI; Kleber Costa de Castro Pires, TSAIII

IAnestesiologista da Santa Casa de Belo Horizonte
IIME3 do CET/SBA Santa Casa de Belo Horizonte
IIIResponsável pelo CET/SBA Santa Casa de Belo Horizonte

Correspondence to




BACKGROUND AND OBJECTIVES: Single ventricle is a rare abnormality, affecting 1% of the patients with congenital cardiopathy. Only 11 cases of patients with unoperated univentricular heart older than 50 years were reported in the literature. The aim of this report was to describe the anesthetic conduct in a patient with univentricular heart undergoing pacemaker implant.
CASE REPORT: A female patient, 47 years old, with double outlet left ventricle, L-transposition of the great vessels, and pulmonary stenosis, without prior surgical correction, was scheduled for definitive implant of a sequential dual-chamber pacemaker. The ABPM demonstrated second degree atrioventricular block and a mean heart rate of 45 bpm. Preoperative exams showed a hematocrit of 57%, normal coagulation studies, and preserved ventricular function. Monitoring consisted of pulse oxymeter, ECG on DII and V5, IBP, capnograph, and gas analyzer. A temporary transcutaneous pacemaker was available in case of severe bradycardia. Anesthesia was induced with fentanyl (0.25 mg), etomidate (20 mg), and atracurium (35 mg). Four minutes after anesthetic induction, the heart rate decreased to 30 bmp and 1 mg of atropine was administered with reversal of the bradycardia. Anesthesia was maintained with 2.5% sevoflurane, 60% room air, and 40% oxygen. Hemodynamic parameters and oxygen saturation remained stable. The patient was transferred to the intensive care unit in stable condition and extubated at the end of the procedure.
CONCLUSIONS: The anesthetic conduct for pacemaker implant in a 47-year old patient with non-operated double outlet left ventricle and pulmonary stenosis was appropriate, since it allowed the procedure to be performed.

Key Words: DISEASES. Congenital: single ventricle; SURGERY, Cardiac: pacemaker implant.


JUSTIFICATIVA Y OBJETIVOS: Ventrículo único es una anormalidad rara encontrada en aproximadamente 1% de los pacientes con cardiopatía congénita. Solamente 11 casos de pacientes con ventrículo único no operado y edad por encima de los 50 años, fueron relatados en la literatura. Este trabajo tiene el objetivo de describir la conducta anestésica en paciente con ventrículo único para implante de marcapaso.
RELATO DEL CASO: Paciente del sexo femenino, 47 años, con doble vía de entrada del ventrículo izquierdo, L-transposición de grandes arterias y estenosis subpulmonar, sin corrección quirúrgica previa, se marcó consulta para implante de marcapaso cardíaco definitivo secuencial de dos cámaras. En el MAPA presentaba bloqueo atrioventricular de segundo grado y una frecuencia cardiaca promedio de 45bpm. Los exámenes preoperatorios mostraban hematócrito de 57%, coagulograma normal, función ventricular preservada. La monitorización constó de oxímetro de pulso, ECG en las derivaciones DII y V5, PIA, capnógrafo y analizador de gases. Un marcapaso temporal transcutáneo quedó a disposición para el caso de bradicardia intensa. La anestesia se indujo con fentanil (0.25 mg), etomidato (20 mg) y atracurio (35 mg). Cuatro minutos después de la inducción, la frecuencia cardiaca disminuyó para 30bpm siendo administrado 1 mg de atropina, con reversión de la bradicardia. La anestesia se mantuvo con sevoflurano a 2.5%, aire 60% y oxígeno 40%. El estado hemodinámico y la saturación de oxígeno permanecieron estables. La paciente fue llevada a la unidad de terapia intensiva estable y extubada al final del procedimiento.
CONCLUSIONES: La conducta anestésica para implante de marcapaso en paciente de 47 años con doble vía de entrada del ventrículo izquierdo y estenosis subpulmonar no operada, fue adecuada, ya que permitió la realización del procedimiento indicado.




Single ventricle is a relatively rare abnormality, affecting of about 1% of the patients with congenital cardiopathy. The presence of subpulmonary stenosis protects the patient against the development of pulmonary hypertension. It has a survival rate of approximately 30% in the first year of life 1 and unoperated patients have a median survival of 14 years 2. Most of the patients are candidates for Fontan procedure or other surgical correction because the prognosis without surgical repair is guarded, and survival to adulthood in these conditions is very rare. Only 11 cases of unoperated univentricular heart in patients older than 50 years have been reported up to now 3. The aim of this report was to describe the anesthetic conduct in a 47 years old patient with unoperated univentricular heart with indication for pacemaker implant.



A female patient, 47 years old, with double outlet left ventricle, ventriculoarterial discordance (L-transposition of the great vessels), and subpulmonary stenosis was scheduled for implant of a definitive, dual-chamber sequential pacemaker. She reported that her symptoms started when she was 21 years old, when she complained of cyanosis and decreased tolerance to efforts. At that time, surgical correction was not mentioned. A 24-hour preoperative ECG as an outpatient showed a second degree atrioventricular block, with a heart rate of about 45 beats per minute (bpm). The echocardiogram revealed a double outlet left ventricle, moderate subpulmonary stenosis, mild pulmonary hypertension, left atrioventricular valve with moderate regurgitation, and preserved ventricular function. Preoperative exams showed hemoglobin of 18.9 g.dL-1, hematocrit 57%, platelet count 205,000/mL, serum potassium 4.1 mmol/L, BUN 28 mg.dL-1, and serum creatinine 0.9 mg.dL-1. Coagulation studies were normal. At the day of the procedure she presented cyanosis, dyspnea, and clubbing of the fingers. Cardiac auscultation revealed a holosystolic murmur throughout the precordium and bilateral lung rales. In the surgical room, the pulse oxymeter showed saturation (SpO2) of 89%, and the electrocardiogram (DII and V5 derivations) demonstrated a second degree atrioventricular block with a mean heart rate of 45 bpm. Blood pressure in the left radial artery was 85 mmHg and stable. Anesthesia was induced with fentanyl (0.25 mg), etomidate (20 mg), and atracurium (35 mg). The patient was intubated and mechanical ventilation was adjusted to maintain a PET CO2 of 33 mmHg in the capnograph. The peak inspiratory pressure was maintained between 18 and 24 cmH20, with a tidal volume of 550 mL. Four minutes after anesthetic induction, the patient presented bradycardia (30 bmp) that was reversed with atropine (1 mg). Anesthesia was maintained with 2.5% sevoflurane, room air 60%, and oxygen 40%. Hemodynamic parameters and SpO2 remained stable. The patient was extubated in the surgical room after the relationship T1/T4 in the train of four achieved 0.9, and she was transferred to the intensive care unit.



Other reports of anesthesia in patients with single ventricle were found, but not in adults with advanced age 4-14. There are no reports of anesthesia in adult patients with unoperated univentricular heart.

In patients with some type of single ventricle, the clinical manifestations and the prognosis are, for the most part, related to the degree of pulmonary stenosis and to the level of pulmonary vascular resistance. In the absence of pulmonary stenosis, there is a large left to right shunt, with an increase in pulmonary blood flow and volume overload of the single ventricle, leading to heart failure and death in the majority of unoperated patients 3. With moderate pulmonary stenosis, the pulmonary blood flow is almost normal, leading to heart failure or not, mild arterial systemic desaturation, and almost no degree of pulmonary arteriolar disease. Longer-term survival is possible with adequate oxygenation and balanced ventricular volumetric load.

There are no reports of long-term survival of patients with right or undetermined type single ventricle. The perspective of survival is greater in patients with the following associations: single ventricle with left ventricular morphology (this allows the preferential flow of oxygenated blood from the left atrium to the aorta); transposition of the great vessels without flow obstruction; atrioventricular valve working properly and moderate obstruction to the pulmonary flow, which allows enough pulmonary flow to prevent severe cyanosis and avoids ventricular overload 15.

Patients with the "ideal" anatomy (single ventricle with left ventricular morphology), with "well-balanced" circulation (some degree of pulmonary stenosis to avoid excessive pulmonary blood flow), and with good ventricular function, can survive with minimal symptomatology and a good capacity for physical exercises 16. Cardiac arrhythmias are the most frequent complications among patients who survive longer.

The patient presented here apparently had this association of alterations, which allowed her to reach the age of 47 years without surgical correction. The main concern was to keep her stable during the procedure, maintaining hemodynamic parameters stable, and avoiding the development of pulmonary hypertension and reflex hypoxic pulmonary vascoconstriction.

An air-oxygen mixture to obtain 50% oxygen concentration was considered adequate, since higher oxygen concentrations could cause pulmonary vasoconstriction. Hypocapnia was avoided for the same reason.

Sevoflurane (1 MAC) was chosen because it seems to offer more cardiovascular stability than other inhalational anesthetics 17. Higher doses of sevoflurane were avoided to decrease the inhibition of the reflex hypoxic pulmonary vasoconstriction.

General anesthesia usually causes vasodilation and a reduction in inotropism or chronotropism. Therefore, a transcutaneous pacemaker was available in case the patient developed severe bradycardia. Due to the anatomy of the patient, implant of a central venous catheter could not be useful and catheterization of the pulmonary artery was probably not possible.

The anesthetic conduct adopted was adequate, allowing the completion of the procedure scheduled.



01. Samanek M - Children with congenital heart disease: probability of natural survival. Pediatr Cardiol, 1992;13:152-158.        [ Links ]

02. Moodie DS, Ritter DG, Tajik AJ et al - Long-term follow-up in the unoperated univentricular heart. Am J Cardiol, 1984;53:1124-1128.        [ Links ]

03. Restaino G, Dirksen MS, de Roos A - Long-term survival in a case of unoperated single ventricle. Int J Cardiovasc Imaging, 2004;20:221-225.        [ Links ]

04. Alon E, Baumann H - Anesthesiologic management of cesarean section in a patient with transposition of the great vessels. Reg Anaesth, 1988;11:28-31.        [ Links ]

05. Uchida K, Ando T, Okuda C - Anesthetic management of an infant with a single ventricle (asplenia syndrome) for non-cardiac surgery. Masui, 1992;41:1793-1797.        [ Links ]

06. Ahmad S, Lichtenthal P - Anesthetic management of a patient with a single ventricle and modified Fontan procedure. J Cardiothorac Vasc Anesth, 1993;7:727-729.        [ Links ]

07. Zavisca FG, Johnson MD, Holubec JT et al - General anesthesia for cesarean section in a parturient with a single ventricle and pulmonary atresia. J Clin Anesth, 1993;5:315-320.        [ Links ]

08. del Pozo D, Sala-Blanch X, Fita G et al - Epidural anesthesia in a patient with single ventricle. Reg Anesth, 1995;20:452-454.        [ Links ]

09. Peng TC, Chuah EC, Tan PP - Epidural anesthesia for emergency cesarean section in a patient with single ventricle and aortic stenosis. Acta Anaesthesiol Sin, 1997;35:39-44.        [ Links ]

10. Ishige A, Ishikawa S, Uchida T et al - Anesthetic management of an infant with asplenia and single atrium single ventricle undergoing ear tube surgery for otitis media: a case report. Masui, 2005;54:304-307.        [ Links ]

11. Sparks JW, Seefelder C, Shamberger RC et al - The perioperative management of a patient with complex single ventricle physiology and pheochromocytoma. Anesth Analg, 2005;100:972-975.        [ Links ]

12. Schummer W, Schummer C, Schleussner E et al. Uncorrected transposition of the great arteries and large ventricular septum defect perioperative management of a cesarean section. Anaesthesist, 2005;54:333-340.        [ Links ]

13. Dubois L, Belkacem H, Berl M et al. Single ventricle and obstetric anaesthesia: two cases report. Ann Fr Anesth Reanim, 2005; 22:50-53.        [ Links ]

14. Tjeuw M, Fong J - Anaesthetic management of a patient with a single ventricle and phaeochromocytoma. Anaesth Intensive Care, 1990;18:567-569.        [ Links ]

15. Hager A, Kaemmerer H, Eicken A et al - Long-term survival of patients with univentricular heart not treated surgically. J Thorac Cardiovasc Surg, 2002;123:1214-1217.        [ Links ]

16. Ammash NM, Warnes CA - Survival into adulthood of patients with unoperated single ventricle. Am J Cardiol, 1996;77:542-544.        [ Links ]

17. Ebert TJ, Harkin CP, Muzi M - Cardiovascular responses to sevoflurane: a review. Anesth Analg, 1995;81:(Suppl2):S11-S22.        [ Links ]



Correspondence to:
Dr. Adriano Bechara de Souza Hobaika
Rua Desembargador Jorge Fontana, 214/2502
30320-670 Belo Horizonte, MG

Submitted from 13 de março de 2006
Accepted para publicação em 23 de outubro de 2006



* Received from da Santa Casa de Belo Horizonte, Belo Horizonte, MG

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